Human MitoCarta3.0: 1136 mitochondrial genes

The MitoCarta3.0 human inventory is a collection of 1136 nuclear and mtDNA genes encoding proteins with strong support of mitochondrial localization. This table provides a summary of evidence supporting mitochondrial localization, the protein distribution across 14 tissues, as well as annotation of biological pathways (based on manual literature review) and sub-compartment (based on annotator's "best guess" integrating sequence features, experimental data, literature, and pathway context; note all subunits of protein complexes are assigned the same compartment). The genes are ordered by strength of mitochondrial evidence. More complete information is available for download in file Human.MitoCarta3.0.xls. See also MitoCarta3.0 home for a description of the project and MitoCarta3.0 documentation for information on the columns below.

SymbolDescriptionSynonymsMaestro scoreEvidenceSub-compartmentMitoPathwaysTissues
CYC1cytochrome ­c1MC3DN6, UQCR4, P08574, gene:153743literature, APEX_IMS, APEX_matrix, targetP signal, yeast mito homolog++, Rickettsial homolog, mito protein domain+, induction, coexpression++, MS/MS++MIMOXPHOS > Complex III > CIII subunits;
Metabolism > Metals and cofactors > Heme-containing proteins;
Metabolism > Electron carriers > Cytochromes;
OXPHOS > OXPHOS subunits
all 14
SDHBsuccinate ­dehydrogenase ­complex ­iron ­sulfur ­subunit ­BCWS2, IP, PGL4, SDH, SDH1, SDH2, SDHIP, P21912, gene:639043literature, APEX_matrix, targetP signal+, yeast mito homolog, Rickettsial homolog, mito protein domain+, induction, coexpression++, MS/MS++MIMOXPHOS > Complex II > CII subunits;
Metabolism > Carbohydrate metabolism > TCA cycle;
Metabolism > Metals and cofactors > Fe-S-containing proteins;
OXPHOS > OXPHOS subunits
all 14
COQ7coenzyme ­Q7, ­hydroxylaseCAT5, CLK-1, CLK1, COQ10D8, Q99807, gene:1022943literature, targetP signal+, yeast mito homolog++, Rickettsial homolog, mito protein domain+, coexpression++, MS/MS++MIMMetabolism > Metals and cofactors > Coenzyme Q metabolismall 14
SDHAsuccinate ­dehydrogenase ­complex ­flavoprotein ­subunit ­ACMD1GG, FP, PGL5, SDH1, SDH2, SDHF, P31040, gene:638942literature, APEX_matrix, targetP signal+, yeast mito homolog++, Rickettsial homolog, mito protein domain, induction, coexpression++, MS/MS++MIMOXPHOS > Complex II > CII subunits;
Metabolism > Carbohydrate metabolism > TCA cycle;
OXPHOS > OXPHOS subunits
all 14
UQCRC1ubiquinol-cytochrome ­c ­reductase ­core ­protein ­1D3S3191, QCR1, UQCR1, P31930, gene:738442literature, APEX_matrix, targetP signal+, yeast mito homolog+, Rickettsial homolog, mito protein domain+, induction, coexpression++, MS/MS++MIMProtein import, sorting and homeostasis > Protein import and sorting > Preprotein cleavage;
OXPHOS > Complex III > CIII subunits;
OXPHOS > OXPHOS subunits
all 14
COQ5coenzyme ­Q5, ­methyltransferaseQ5HYK3, gene:8427442APEX_matrix, targetP signal, yeast mito homolog++, Rickettsial homolog, mito protein domain+, induction, coexpression++, MS/MS++MIMMetabolism > Metals and cofactors > Coenzyme Q metabolismall 14
PDHA1pyruvate ­dehydrogenase ­E1 ­subunit ­alpha ­1PDHA, PDHAD, PDHCE1A, PHE1A, P08559, gene:516042literature, APEX_matrix, targetP signal+, yeast mito homolog+, Rickettsial homolog, mito protein domain+, induction, coexpression++, MS/MS++MatrixMetabolism > Carbohydrate metabolism > Pyruvate metabolismall 14
COQ9coenzyme ­Q9C16orf49, COQ10D5, O75208, gene:5701742APEX_matrix, targetP signal+, yeast mito homolog++, Rickettsial homolog, mito protein domain+, induction, coexpression, MS/MS++MIMMetabolism > Metals and cofactors > Coenzyme Q metabolismall 14
MRPL12mitochondrial ­ribosomal ­protein ­L125c5-2, L12mt, MRP-L31/34, MRPL7, MRPL7/L12, RPML12, P52815, gene:618242literature, targetP signal+, yeast mito homolog++, Rickettsial homolog, mito protein domain, induction, coexpression++, MS/MS++MatrixMitochondrial central dogma > mtRNA metabolism > Transcription;
Mitochondrial central dogma > Translation > Mitochondrial ribosome
all 14
ATP5F1DATP ­synthase ­F1 ­subunit ­deltaATP5D, MC5DN5, P30049, gene:51342literature, APEX_matrix, targetP signal, yeast mito homolog++, Rickettsial homolog, mito protein domain+, induction, coexpression++, MS/MS+MIMOXPHOS > Complex V > CV subunits;
OXPHOS > OXPHOS subunits
all 14
COX5Acytochrome ­c ­oxidase ­subunit ­5ACOX, COX-VA, VA, P20674, gene:937742literature, APEX_IMS, APEX_matrix, targetP signal+, yeast mito homolog++, mito protein domain+, induction, coexpression++, MS/MS++MIMOXPHOS > Complex IV > CIV subunits;
OXPHOS > OXPHOS subunits
all 14
ISCA2iron-sulfur ­cluster ­assembly ­2HBLD1, ISA2, MMDS4, c14_5557, Q86U28, gene:12296142APEX_matrix, targetP signal+, yeast mito homolog++, Rickettsial homolog, mito protein domain+, induction, coexpression, MS/MS++MatrixMetabolism > Metals and cofactors > Fe-S cluster biosynthesis;
Metabolism > Metals and cofactors > Fe-S-containing proteins
all 14
PMPCBpeptidase, ­mitochondrial ­processing ­subunit ­betaBeta-MPP, MPP11, MPPB, MPPP52, P-52, O75439, gene:951241literature, APEX_matrix, targetP signal+, yeast mito homolog+, Rickettsial homolog, mito protein domain+, induction, coexpression++, MS/MS++MatrixProtein import, sorting and homeostasis > Protein import and sorting > Preprotein cleavage;
Protein import, sorting and homeostasis > Protein homeostasis > Proteases
all 14
UQCRFS1ubiquinol-cytochrome ­c ­reductase, ­Rieske ­iron-sulfur ­polypeptide ­1MC3DN10, RIP1, RIS1, RISP, UQCR5, P47985, gene:738640literature, APEX_matrix, targetP signal, yeast mito homolog++, Rickettsial homolog, mito protein domain+, coexpression++, MS/MS++MIMOXPHOS > Complex III > CIII subunits;
Metabolism > Metals and cofactors > Fe-S-containing proteins;
OXPHOS > OXPHOS subunits
all 14
ATP5F1AATP ­synthase ­F1 ­subunit ­alphaATP5A, ATP5A1, ATP5AL2, ATPM, COXPD22, HEL-S-123m, MC5DN4, MOM2, OMR, ORM, hATP1, P25705, gene:49840literature, APEX_matrix, targetP signal+, yeast mito homolog++, Rickettsial homolog, mito protein domain, induction, coexpression++, MS/MS++MIMOXPHOS > Complex V > CV subunits;
OXPHOS > OXPHOS subunits
all 14
OGDHoxoglutarate ­dehydrogenaseAKGDH, E1k, KGD1, OGDC, OGDH2, Q02218, gene:496740literature, APEX_matrix, targetP signal, yeast mito homolog+, Rickettsial homolog, mito protein domain+, induction, coexpression++, MS/MS++MatrixMetabolism > Carbohydrate metabolism > TCA cycleall 14
PDHBpyruvate ­dehydrogenase ­E1 ­subunit ­betaPDHBD, PDHE1-B, PDHE1B, PHE1B, P11177, gene:516240literature, targetP signal, yeast mito homolog+, Rickettsial homolog, mito protein domain+, induction, coexpression++, MS/MS++MatrixMetabolism > Carbohydrate metabolism > Pyruvate metabolismall 14
UQCRC2ubiquinol-cytochrome ­c ­reductase ­core ­protein ­2MC3DN5, QCR2, UQCR2, P22695, gene:738540literature, APEX_matrix, targetP signal, yeast mito homolog+, Rickettsial homolog, mito protein domain+, induction, coexpression++, MS/MS++MIMProtein import, sorting and homeostasis > Protein import and sorting > Preprotein cleavage;
OXPHOS > Complex III > CIII subunits;
OXPHOS > OXPHOS subunits
all 14
SDHDsuccinate ­dehydrogenase ­complex ­subunit ­DCBT1, CII-4, CWS3, PGL, PGL1, QPs3, SDH4, cybS, O14521, gene:639240literature, targetP signal, yeast mito homolog++, Rickettsial homolog, mito protein domain+, induction, coexpression++, MS/MS+MIMOXPHOS > Complex II > CII subunits;
Metabolism > Carbohydrate metabolism > TCA cycle;
Metabolism > Metals and cofactors > Heme-containing proteins;
OXPHOS > OXPHOS subunits
all 14
MRPS35mitochondrial ­ribosomal ­protein ­S35HDCMD11P, MDS023, MRP-S28, MRPS28, P82673, gene:6048839literature, APEX_matrix, targetP signal, yeast mito homolog++, mito protein domain+, induction, coexpression++, MS/MS++MatrixMitochondrial central dogma > Translation > Mitochondrial ribosomeall 14
UQCRQubiquinol-cytochrome ­c ­reductase ­complex ­III ­subunit ­VIIMC3DN4, QCR8, QP-C, QPC, UQCR7, O14949, gene:2708939literature, APEX_IMS, targetP signal, yeast mito homolog++, mito protein domain+, induction, coexpression++, MS/MS++MIMOXPHOS > Complex III > CIII subunits;
OXPHOS > OXPHOS subunits
all 14
MRPL53mitochondrial ­ribosomal ­protein ­L53L53MT, Q96EL3, gene:11654039literature, APEX_matrix, targetP signal, yeast mito homolog++, mito protein domain+, induction, coexpression++, MS/MS++MatrixMitochondrial central dogma > Translation > Mitochondrial ribosomeall 14
DBTdihydrolipoamide ­branched ­chain ­transacylase ­E2BCATE2, BCKAD-E2, BCKADE2, BCKDH-E2, BCOADC-E2, E2, E2B, P11182, gene:162939literature, APEX_matrix, targetP signal+, yeast mito homolog+, Rickettsial homolog, mito protein domain+, induction, coexpression+, MS/MS++MatrixMetabolism > Amino acid metabolism > BCAA metabolism;
Metabolism > Amino acid metabolism > BCAA dehydrogenase complex
all 14
PDK4pyruvate ­dehydrogenase ­kinase ­4Q16654, gene:516638literature, targetP signal, yeast mito homolog++, Rickettsial homolog, mito protein domain+, induction, coexpression, MS/MS++MIMMetabolism > Carbohydrate metabolism > Pyruvate metabolismcerebrum, cerebellum, brainstem, spinalcord, kidney, liver, heart, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, testis
MDH2malate ­dehydrogenase ­2EIEE51, M-MDH, MDH, MGC:3559, MOR1, P40926, gene:419138literature, APEX_matrix, targetP signal, yeast mito homolog++, Rickettsial homolog, mito protein domain, induction, coexpression++, MS/MS++MatrixMetabolism > Carbohydrate metabolism > Gluconeogenesis;
Metabolism > Carbohydrate metabolism > Malate-aspartate shuttle;
Metabolism > Carbohydrate metabolism > TCA cycle
all 14
MRPS27mitochondrial ­ribosomal ­protein ­S27MRP-S27, S27mt, Q92552, gene:2310738literature, APEX_matrix, targetP signal+, yeast mito homolog++, mito protein domain+, induction, coexpression+, MS/MS++MatrixMitochondrial central dogma > Translation > Mitochondrial ribosomecerebrum, cerebellum, brainstem, spinalcord, kidney, liver, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
CScitrate ­synthaseO75390, gene:143137literature, APEX_matrix, targetP signal, yeast mito homolog++, Rickettsial homolog, mito protein domain, induction, coexpression++, MS/MS++MatrixMetabolism > Carbohydrate metabolism > TCA cycleall 14
GRPEL1GrpE ­like ­1, ­mitochondrialGrpE, HMGE, mt-GrpE#1, Q9HAV7, gene:8027337literature, APEX_matrix, targetP signal+, Rickettsial homolog, mito protein domain+, induction, coexpression++, MS/MS++MatrixProtein import, sorting and homeostasis > Protein import and sorting > Import motor;
Metabolism > Metals and cofactors > Fe-S cluster biosynthesis
all 14
DLATdihydrolipoamide ­S-acetyltransferaseDLTA, E2, PBC, PDC-E2, PDCE2, P10515, gene:173737literature, APEX_matrix, yeast mito homolog+, Rickettsial homolog, mito protein domain+, induction, coexpression++, MS/MS++MatrixMetabolism > Carbohydrate metabolism > Pyruvate metabolismall 14
LRPPRCleucine ­rich ­pentatricopeptide ­repeat ­containingCLONE-23970, GP130, LRP130, LSFC, P42704, gene:1012837literature, APEX_matrix, targetP signal+, yeast mito homolog++, mito protein domain, induction, coexpression++, MS/MS++MatrixMitochondrial central dogma > mtRNA metabolism > mtRNA stability and decay;
Mitochondrial central dogma > Translation
all 14
DLSTdihydrolipoamide ­S-succinyltransferaseDLTS, KGD2, PGL7, P36957, gene:174337literature, APEX_IMS, APEX_matrix, targetP signal, yeast mito homolog+, Rickettsial homolog, mito protein domain+, induction, coexpression++, MS/MS+MatrixMetabolism > Carbohydrate metabolism > TCA cycle;
Metabolism > Amino acid metabolism > Lysine metabolism
all 14
PDHXpyruvate ­dehydrogenase ­complex ­component ­XDLDBP, E3BP, OPDX, PDHXD, PDX1, proX, O00330, gene:805037literature, APEX_matrix, targetP signal, yeast mito homolog+, Rickettsial homolog, mito protein domain+, induction, coexpression++, MS/MS+MatrixMetabolism > Carbohydrate metabolism > Pyruvate metabolismall 14
GFM1G ­elongation ­factor ­mitochondrial ­1COXPD1, EFG, EFG1, EFGM, EGF1, GFM, hEFG1, mtEF-G1, Q96RP9, gene:8547637literature, APEX_matrix, targetP signal, yeast mito homolog++, Rickettsial homolog, mito protein domain, induction, coexpression++, MS/MS++MatrixMitochondrial central dogma > Translation > Translation factorsall 14
MPC2mitochondrial ­pyruvate ­carrier ­2BRP44, SLC54A2, O95563, gene:2587437literature, yeast mito homolog++, mito protein domain+, induction, coexpression++, MS/MS++MIMMetabolism > Carbohydrate metabolism > Gluconeogenesis;
Metabolism > Carbohydrate metabolism > Pyruvate metabolism;
Metabolism > Carbohydrate metabolism > TCA-associated;
Small molecule transport
all 14
NDUFS1NADH:ubiquinone ­oxidoreductase ­core ­subunit ­S1CI-75Kd, CI-75k, MC1DN5, PRO1304, P28331, gene:471936literature, APEX_matrix, targetP signal, Rickettsial homolog, mito protein domain+, induction, coexpression++, MS/MS++MIMOXPHOS > Complex I > CI subunits;
Metabolism > Metals and cofactors > Fe-S-containing proteins;
OXPHOS > OXPHOS subunits
all 14
MRPL46mitochondrial ­ribosomal ­protein ­L46C15orf4, LIECG2, P2ECSL, Q9H2W6, gene:2658936literature, APEX_matrix, targetP signal+, yeast mito homolog++, mito protein domain, induction, coexpression++, MS/MS++MatrixMitochondrial central dogma > Translation > Mitochondrial ribosomecerebrum, cerebellum, spinalcord, kidney, liver, heart, adipose, smallintestine, largeintestine, placenta, testis
ATP5F1EATP ­synthase ­F1 ­subunit ­epsilonATP5E, ATPE, MC5DN3, P56381, gene:51436literature, yeast mito homolog++, mito protein domain+, induction, coexpression++, MS/MS++MIMOXPHOS > Complex V > CV subunits;
OXPHOS > OXPHOS subunits
all 14
SLC25A3solute ­carrier ­family ­25 ­member ­3OK/SW-cl.48, PHC, PTP, Q00325, gene:525036literature, yeast mito homolog++, mito protein domain+, induction, coexpression++, MS/MS++MIMMetabolism > Metals and cofactors > Copper metabolism;
Signaling > Calcium homeostasis > Calcium cycle;
Small molecule transport > SLC25A family
all 14
MRPS23mitochondrial ­ribosomal ­protein ­S23CGI-138, COXPD46, HSPC329, MRP-S23, Q9Y3D9, gene:5164936literature, APEX_matrix, yeast mito homolog++, mito protein domain+, induction, coexpression++, MS/MS++MatrixMitochondrial central dogma > Translation > Mitochondrial ribosomeall 14
FHfumarate ­hydrataseFMRD, HLRCC, HsFH, LRCC, MCL, MCUL1, P07954, gene:227135literature, APEX_matrix, targetP signal+, yeast mito homolog, Rickettsial homolog, mito protein domain, induction, coexpression++, MS/MS++MatrixMetabolism > Carbohydrate metabolism > TCA cycleall 14
PMPCApeptidase, ­mitochondrial ­processing ­subunit ­alphaAlpha-MPP, CLA1, CPD3, INPP5E, P-55, SCAR2, Q10713, gene:2320335literature, APEX_matrix, targetP signal+, yeast mito homolog++, Rickettsial homolog, mito protein domain+, coexpression, MS/MS++MatrixProtein import, sorting and homeostasis > Protein import and sorting > Preprotein cleavage;
Protein import, sorting and homeostasis > Protein homeostasis > Proteases
all 14
ATP5F1BATP ­synthase ­F1 ­subunit ­betaATP5B, ATPMB, ATPSB, HEL-S-271, P06576, gene:50635literature, APEX_matrix, targetP signal+, yeast mito homolog++, Rickettsial homolog, mito protein domain, coexpression++, MS/MS++MIMOXPHOS > Complex V > CV subunits;
OXPHOS > OXPHOS subunits
all 14
SDHAF4succinate ­dehydrogenase ­complex ­assembly ­factor ­4C6orf57, Sdh8, Q5VUM1, gene:13515435yeast mito homolog++, Rickettsial homolog, mito protein domain+, induction, coexpression++, MS/MSMatrixOXPHOS > Complex II > CII assembly factors;
OXPHOS > OXPHOS assembly factors
adipose, testis
UQCR10ubiquinol-cytochrome ­c ­reductase, ­complex ­III ­subunit ­XHSPC051, HSPC119, HSPC151, QCR9, UCCR7.2, UCRC, Q9UDW1, gene:2979635literature, yeast mito homolog++, mito protein domain+, induction, coexpression++, MS/MS++MIMOXPHOS > Complex III > CIII subunits;
OXPHOS > OXPHOS subunits
all 14
ISCA1iron-sulfur ­cluster ­assembly ­1HBLD2, ISA1, MMDS5, hIscA, Q9BUE6, gene:8168935APEX_matrix, targetP signal, yeast mito homolog++, Rickettsial homolog, mito protein domain+, induction, MS/MS++MatrixMetabolism > Metals and cofactors > Fe-S cluster biosynthesis;
Metabolism > Metals and cofactors > Fe-S-containing proteins
cerebrum, cerebellum, brainstem, spinalcord, liver, heart, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
SUCLA2succinate-CoA ­ligase ­ADP-forming ­subunit ­betaA-BETA, A-SCS, LINC00444, MTDPS5, SCS-betaA, Q9P2R7, gene:880335literature, APEX_matrix, targetP signal+, yeast mito homolog+, Rickettsial homolog, mito protein domain, induction, coexpression+, MS/MS++MatrixMetabolism > Carbohydrate metabolism > TCA cycle;
Metabolism > Carbohydrate metabolism > Itaconate metabolism;
Metabolism > Nucleotide metabolism > Nucleotide synthesis and processing
all 14
COQ3coenzyme ­Q3, ­methyltransferaseDHHBMT, DHHBMTASE, UG0215E05, bA9819.1, Q9NZJ6, gene:5180534APEX_matrix, targetP signal, yeast mito homolog++, Rickettsial homolog, mito protein domain+, induction, coexpression+, MS/MS+MIMMetabolism > Metals and cofactors > Coenzyme Q metabolismcerebrum, cerebellum, brainstem, spinalcord, liver, heart, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
IARS2isoleucyl-tRNA ­synthetase ­2, ­mitochondrialCAGSSS, ILERS, Q9NSE4, gene:5569934APEX_matrix, targetP signal+, yeast mito homolog++, Rickettsial homolog, mito protein domain, coexpression++, MS/MS++MatrixMitochondrial central dogma > Translation > mt-tRNA synthetasesall 14
MRPS15mitochondrial ­ribosomal ­protein ­S15DC37, MPR-S15, RPMS15, S15mt, P82914, gene:6496034literature, targetP signal, yeast mito homolog++, Rickettsial homolog, mito protein domain, coexpression++, MS/MS++MatrixMitochondrial central dogma > Translation > Mitochondrial ribosomeall 14
IDH3Aisocitrate ­dehydrogenase ­(NAD(+)) ­3 ­catalytic ­subunit ­alphaP50213, gene:341934APEX_matrix, yeast mito homolog++, Rickettsial homolog, mito protein domain, induction, coexpression++, MS/MS++MatrixMetabolism > Carbohydrate metabolism > TCA cycleall 14
COX11cytochrome ­c ­oxidase ­copper ­chaperone ­COX11COX11P, Q9Y6N1, gene:135334literature, APEX_IMS, targetP signal, yeast mito homolog++, Rickettsial homolog, mito protein domain+, induction, coexpression, MS/MSMIMOXPHOS > Complex IV > CIV assembly factors;
Metabolism > Metals and cofactors > Copper metabolism;
OXPHOS > OXPHOS assembly factors
cerebrum, cerebellum, spinalcord, skeletalmuscle, largeintestine, placenta
ETFDHelectron ­transfer ­flavoprotein ­dehydrogenaseETFQO, MADD, Q16134, gene:211034literature, APEX_matrix, yeast mito homolog++, mito protein domain+, induction, coexpression++, MS/MS++MIMMetabolism > Lipid metabolism > Fatty acid oxidation;
Metabolism > Amino acid metabolism > BCAA metabolism;
Metabolism > Amino acid metabolism > Lysine metabolism;
Metabolism > Amino acid metabolism > Glycine metabolism;
Metabolism > Metals and cofactors > Fe-S-containing proteins;
Metabolism > Vitamin metabolism > Choline and betaine metabolism;
Metabolism > Electron carriers > Q-linked reactions, other
all 14
TIMM10translocase ­of ­inner ­mitochondrial ­membrane ­10TIM10, TIM10A, TIMM10A, P62072, gene:2651934literature, APEX_IMS, yeast mito homolog++, mito protein domain+, induction, coexpression++, MS/MS++MIMProtein import, sorting and homeostasis > Protein homeostasis > Chaperones;
Protein import, sorting and homeostasis > Protein import and sorting
cerebrum, cerebellum, brainstem, spinalcord, liver, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
MRPL34mitochondrial ­ribosomal ­protein ­L34L34mt, Q9BQ48, gene:6498134literature, APEX_matrix, targetP signal, yeast mito homolog++, Rickettsial homolog, mito protein domain, induction, coexpression++, MS/MSMatrixMitochondrial central dogma > Translation > Mitochondrial ribosomecerebrum, cerebellum, brainstem, spinalcord, kidney, liver, heart, adipose, smallintestine, largeintestine, stomach, placenta, testis
MRPL2mitochondrial ­ribosomal ­protein ­L2CGI-22, MRP-L14, RPML14, Q5T653, gene:5106934literature, APEX_matrix, targetP signal, yeast mito homolog++, Rickettsial homolog, mito protein domain, induction, coexpression++, MS/MS++MatrixMitochondrial central dogma > Translation > Mitochondrial ribosomespinalcord, liver, heart, adipose, smallintestine, largeintestine, placenta, testis
BCKDHAbranched ­chain ­keto ­acid ­dehydrogenase ­E1 ­subunit ­alphaBCKDE1A, MSU, MSUD1, OVD1A, P12694, gene:59334literature, APEX_matrix, targetP signal+, yeast mito homolog+, Rickettsial homolog, mito protein domain+, coexpression+, MS/MS++MIMMetabolism > Amino acid metabolism > BCAA metabolism;
Metabolism > Amino acid metabolism > BCAA dehydrogenase complex
cerebrum, spinalcord, kidney, liver, heart, skeletalmuscle, adipose, largeintestine, stomach, placenta, testis
UQCRHubiquinol-cytochrome ­c ­reductase ­hinge ­proteinQCR6, UQCR8, P07919, gene:738834literature, APEX_IMS, yeast mito homolog++, mito protein domain+, induction, coexpression++, MS/MS++MIMOXPHOS > Complex III > CIII subunits;
OXPHOS > OXPHOS subunits
all 14
HIGD2AHIG1 ­hypoxia ­inducible ­domain ­family ­member ­2ARCF1b, Q9BW72, gene:19228634APEX_IMS, yeast mito homolog++, mito protein domain+, induction, coexpression++, MS/MS++MIMOXPHOS > OXPHOS assembly factors;
OXPHOS > Respirasome assembly
all 14
ATP5POATP ­synthase ­peripheral ­stalk ­subunit ­OSCPATP5O, ATPO, HMC08D05, OSCP, P48047, gene:53934literature, APEX_matrix, targetP signal, yeast mito homolog++, Rickettsial homolog, mito protein domain+, induction, MS/MS++MIMOXPHOS > Complex V > CV subunits;
OXPHOS > OXPHOS subunits
all 14
ECHS1enoyl-CoA ­hydratase, ­short ­chain ­1ECHS1D, SCEH, P30084, gene:189233literature, APEX_matrix, targetP signal+, yeast mito homolog+, Rickettsial homolog, mito protein domain, induction, coexpression+, MS/MS++MatrixMetabolism > Lipid metabolism > Fatty acid oxidation;
Metabolism > Amino acid metabolism > BCAA metabolism;
Metabolism > Amino acid metabolism > Lysine metabolism
all 14
LETMD1LETM1 ­domain ­containing ­11110019O13Rik, HCCR, HCCR-1, HCCR-2, HCCR1, HCCR2, HCRR-2, SLC55A3, Q6P1Q0, gene:2587533GFP, targetP signal+, yeast mito homolog+, mito protein domain+, induction, coexpression, MS/MS++MembraneSmall molecule transportcerebrum, cerebellum, brainstem, spinalcord, kidney, liver, adipose, smallintestine, largeintestine, placenta, testis
COX6A1cytochrome ­c ­oxidase ­subunit ­6A1CMTRID, COX6A, COX6AL, P12074, gene:133733literature, targetP signal+, yeast mito homolog+, mito protein domain+, induction, coexpression, MS/MS++MIMOXPHOS > Complex IV > CIV subunits;
OXPHOS > OXPHOS subunits
all 14
COX15cytochrome ­c ­oxidase ­assembly ­homolog ­COX15CEMCOX2, Q7KZN9, gene:135533literature, targetP signal, yeast mito homolog++, Rickettsial homolog, mito protein domain+, coexpression++, MS/MSMIMOXPHOS > Complex IV > CIV assembly factors;
Metabolism > Metals and cofactors > Heme synthesis and processing;
OXPHOS > OXPHOS assembly factors
cerebrum, brainstem, spinalcord, kidney, liver, adipose, smallintestine, largeintestine, stomach, placenta, testis
AFG3L2AFG3 ­like ­matrix ­AAA ­peptidase ­subunit ­2OPA12, SCA28, SPAX5, Q9Y4W6, gene:1093933literature, APEX_matrix, targetP signal, Rickettsial homolog, mito protein domain+, induction, coexpression++, MS/MS++MIMProtein import, sorting and homeostasis > Protein homeostasis > Proteasesall 14
HADHAhydroxyacyl-CoA ­dehydrogenase ­trifunctional ­multienzyme ­complex ­subunit ­alphaECHA, GBP, HADH, LCEH, LCHAD, MTPA, TP-ALPHA, P40939, gene:303033literature, APEX_matrix, targetP signal+, yeast mito homolog+, Rickettsial homolog, mito protein domain, coexpression++, MS/MS++MIMMetabolism > Lipid metabolism > Fatty acid oxidation;
Metabolism > Lipid metabolism > Cardiolipin synthesis;
Metabolism > Amino acid metabolism > BCAA metabolism
all 14
ETFAelectron ­transfer ­flavoprotein ­subunit ­alphaEMA, GA2, MADD, P13804, gene:210833literature, APEX_matrix, targetP signal, yeast mito homolog++, mito protein domain, induction, coexpression++, MS/MS++MatrixMetabolism > Lipid metabolism > Fatty acid oxidation;
Metabolism > Amino acid metabolism > BCAA metabolism;
Metabolism > Amino acid metabolism > Lysine metabolism;
Metabolism > Amino acid metabolism > Glycine metabolism;
Metabolism > Vitamin metabolism > Choline and betaine metabolism;
Metabolism > Electron carriers > Q-linked reactions, other
all 14
NDUFS7NADH:ubiquinone ­oxidoreductase ­core ­subunit ­S7CI-20, CI-20KD, MC1DN3, MY017, PSST, O75251, gene:37429133literature, APEX_matrix, targetP signal+, Rickettsial homolog, mito protein domain, induction, coexpression++, MS/MS++MIMOXPHOS > Complex I > CI subunits;
Metabolism > Metals and cofactors > Fe-S-containing proteins;
OXPHOS > OXPHOS subunits
all 14
CPT2carnitine ­palmitoyltransferase ­2CPT1, CPTASE, IIAE4, P23786, gene:137632literature, APEX_matrix, targetP signal+, yeast mito homolog++, mito protein domain, induction, coexpression+, MS/MS++MIMMetabolism > Lipid metabolism > Fatty acid oxidation;
Metabolism > Metals and cofactors > Carnitine synthesis and transport;
Metabolism > Metals and cofactors > Carnitine shuttle
all 14
BCKDHBbranched ­chain ­keto ­acid ­dehydrogenase ­E1 ­subunit ­betaBCKDE1B, BCKDH E1-beta, E1B, P21953, gene:59432literature, APEX_matrix, targetP signal, yeast mito homolog+, Rickettsial homolog, mito protein domain+, coexpression+, MS/MS++MIMMetabolism > Amino acid metabolism > BCAA metabolism;
Metabolism > Amino acid metabolism > BCAA dehydrogenase complex
cerebrum, cerebellum, brainstem, spinalcord, liver, heart, skeletalmuscle, adipose, largeintestine, stomach, placenta, testis
IDH3Bisocitrate ­dehydrogenase ­(NAD(+)) ­3 ­non-catalytic ­subunit ­betaRP46, O43837, gene:342032literature, APEX_matrix, targetP signal+, yeast mito homolog+, Rickettsial homolog, mito protein domain, induction, coexpression, MS/MS++MatrixMetabolism > Carbohydrate metabolism > TCA cycleall 14
LARS2leucyl-tRNA ­synthetase ­2, ­mitochondrialHLASA, LEURS, PRLTS4, mtLeuRS, Q15031, gene:2339532literature, APEX_matrix, targetP signal, yeast mito homolog++, Rickettsial homolog, mito protein domain, coexpression++, MS/MS++MatrixMitochondrial central dogma > Translation > mt-tRNA synthetasesall 14
ACADSacyl-CoA ­dehydrogenase ­short ­chainACAD3, SCAD, P16219, gene:3532literature, APEX_matrix, targetP signal+, mito protein domain+, induction, coexpression++, MS/MS++MatrixMetabolism > Lipid metabolism > Fatty acid oxidationall 14
LETM1leucine ­zipper ­and ­EF-hand ­containing ­transmembrane ­protein ­1SLC55A1, O95202, gene:395432literature, APEX_IMS, APEX_matrix, targetP signal, yeast mito homolog+, mito protein domain+, coexpression++, MS/MS++MIMSignaling > Calcium homeostasis > Calcium cycleall 14
ATP5MEATP ­synthase ­membrane ­subunit ­eATP5I, ATP5K, P56385, gene:52132literature, APEX_IMS, yeast mito homolog++, mito protein domain+, coexpression++, MS/MS++MIMOXPHOS > Complex V > CV subunits;
OXPHOS > OXPHOS subunits;
Mitochondrial dynamics and surveillance > Cristae formation
all 14
OPA1OPA1 ­mitochondrial ­dynamin ­like ­GTPaseBERHS, MGM1, MTDPS14, NPG, NTG, largeG, O60313, gene:497632literature, APEX_IMS, targetP signal, yeast mito homolog++, mito protein domain, induction, coexpression++, MS/MS++MIMMitochondrial dynamics and surveillance > Fusion;
Mitochondrial dynamics and surveillance > Cristae formation
all 14
AUHAU ­RNA ­binding ­methylglutaconyl-CoA ­hydrataseQ13825, gene:54932literature, APEX_matrix, yeast mito homolog+, Rickettsial homolog, mito protein domain, induction, coexpression++, MS/MS++MatrixMetabolism > Carbohydrate metabolism > Itaconate metabolism;
Metabolism > Amino acid metabolism > BCAA metabolism
all 14
SUCLG1succinate-CoA ­ligase ­GDP/ADP-forming ­subunit ­alphaGALPHA, MTDPS9, SUCLA1, P53597, gene:880232yeast mito homolog+, Rickettsial homolog, mito protein domain, induction, coexpression++, MS/MS++MatrixMetabolism > Carbohydrate metabolism > TCA cycle;
Metabolism > Carbohydrate metabolism > Itaconate metabolism;
Metabolism > Nucleotide metabolism > Nucleotide synthesis and processing
all 14
NDUFV2NADH:ubiquinone ­oxidoreductase ­core ­subunit ­V2CI-24k, MC1DN7, P19404, gene:472932literature, APEX_matrix, targetP signal+, Rickettsial homolog, induction, coexpression++, MS/MS++MIMOXPHOS > Complex I > CI subunits;
Metabolism > Metals and cofactors > Fe-S-containing proteins;
OXPHOS > OXPHOS subunits
all 14
COQ6coenzyme ­Q6, ­monooxygenaseCGI-10, CGI10, COQ10D6, Q9Y2Z9, gene:5100432literature, APEX_matrix, targetP signal+, yeast mito homolog++, Rickettsial homolog, mito protein domain, induction, coexpression+, MS/MS+MIMMetabolism > Metals and cofactors > Coenzyme Q metabolismcerebrum, cerebellum, spinalcord, kidney, liver, heart, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
MRPL43mitochondrial ­ribosomal ­protein ­L43L43mt, MRP-L43, bMRP36a, Q8N983, gene:8454531literature, targetP signal+, yeast mito homolog++, mito protein domain, induction, coexpression, MS/MS++MatrixMitochondrial central dogma > Translation > Mitochondrial ribosomeall 14
ABHD11abhydrolase ­domain ­containing ­11PP1226, WBSCR21, Q8NFV4, gene:8345131literature, APEX_matrix, targetP signal+, yeast mito homolog++, Rickettsial homolog, mito protein domain, coexpression+, MS/MS++MatrixMetabolism > Carbohydrate metabolism > TCA-associatedcerebrum, brainstem, spinalcord, kidney, liver, heart, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
ATP5PFATP ­synthase ­peripheral ­stalk ­subunit ­F6ATP5, ATP5A, ATP5J, ATPM, CF6, F6, P18859, gene:52231literature, APEX_IMS, targetP signal+, mito protein domain+, induction, coexpression++, MS/MS++MIMOXPHOS > Complex V > CV subunits;
OXPHOS > OXPHOS subunits
all 14
NDUFB8NADH:ubiquinone ­oxidoreductase ­subunit ­B8ASHI, CI-ASHI, MC1DN32, O95169, gene:471431literature, APEX_IMS, APEX_matrix, targetP signal+, mito protein domain+, induction, coexpression++, MS/MS++MIMOXPHOS > Complex I > CI subunits;
OXPHOS > OXPHOS subunits
all 14
LONP1lon ­peptidase ­1, ­mitochondrialCODASS, LON, LONP, LonHS, PIM1, PRSS15, hLON, P36776, gene:936131literature, APEX_matrix, targetP signal+, yeast mito homolog+, Rickettsial homolog, mito protein domain, induction, coexpression, MS/MS++MatrixProtein import, sorting and homeostasis > Protein homeostasis > Proteasesall 14
DLDdihydrolipoamide ­dehydrogenaseDLDD, DLDH, E3, GCSL, LAD, OGDC-E3, PHE3, P09622, gene:173831literature, APEX_matrix, targetP signal, yeast mito homolog, Rickettsial homolog, mito protein domain, induction, coexpression++, MS/MS++MatrixMetabolism > Carbohydrate metabolism > Pyruvate metabolism;
Metabolism > Carbohydrate metabolism > TCA cycle;
Metabolism > Amino acid metabolism > BCAA metabolism;
Metabolism > Amino acid metabolism > BCAA dehydrogenase complex;
Metabolism > Amino acid metabolism > Lysine metabolism;
Metabolism > Amino acid metabolism > Glycine metabolism;
Metabolism > Amino acid metabolism > Glycine cleavage system
all 14
AIFM1apoptosis ­inducing ­factor ­mitochondria ­associated ­1AIF, AUNX1, CMT2D, CMTX4, COWCK, COXPD6, DFNX5, NADMR, NAMSD, PDCD8, SEMDHL, O95831, gene:913131literature, APEX_IMS, targetP signal, Rickettsial homolog, mito protein domain, induction, coexpression++, MS/MS++MIMProtein import, sorting and homeostasis > Protein import and sorting > MIA40;
OXPHOS > Complex I > CI assembly factors;
OXPHOS > OXPHOS assembly factors;
Mitochondrial dynamics and surveillance > Apoptosis
all 14
ECHDC3enoyl-CoA ­hydratase ­domain ­containing ­3Q96DC8, gene:7974631targetP signal+, yeast mito homolog+, Rickettsial homolog, mito protein domain, induction, coexpression, MS/MS++Matrixnonespinalcord, kidney, liver, heart, adipose, largeintestine, stomach, placenta, testis
APOOLapolipoprotein ­O ­likeCXorf33, FAM121A, MICOS27, Mic27, UNQ8193, Q6UXV4, gene:13932231literature, yeast mito homolog, mito protein domain+, induction, coexpression++, MS/MS++MIMMitochondrial dynamics and surveillance > Cristae formation > MICOS complexall 14
MRPL10mitochondrial ­ribosomal ­protein ­L10L10MT, MRP-L10, MRP-L8, MRPL8, RPML8, Q7Z7H8, gene:12499531literature, APEX_matrix, targetP signal, yeast mito homolog++, Rickettsial homolog, mito protein domain, coexpression+, MS/MS++MatrixMitochondrial central dogma > Translation > Mitochondrial ribosomeall 14
MRRFmitochondrial ­ribosome ­recycling ­factorMRFF, MTRRF, RRF, Q96E11, gene:9239930literature, APEX_matrix, targetP signal+, yeast mito homolog++, Rickettsial homolog, mito protein domain, coexpression, MS/MS++MatrixMitochondrial central dogma > Translation > Translation factorsall 14
NDUFS8NADH:ubiquinone ­oxidoreductase ­core ­subunit ­S8CI-23k, CI23KD, MC1DN2, TYKY, O00217, gene:472830literature, APEX_matrix, targetP signal, Rickettsial homolog, mito protein domain, induction, coexpression++, MS/MS++MIMOXPHOS > Complex I > CI subunits;
Metabolism > Metals and cofactors > Fe-S-containing proteins;
OXPHOS > OXPHOS subunits
all 14
ACADMacyl-CoA ­dehydrogenase ­medium ­chainACAD1, MCAD, MCADH, P11310, gene:3430literature, APEX_matrix, targetP signal, mito protein domain+, induction, coexpression++, MS/MS++MatrixMetabolism > Lipid metabolism > Fatty acid oxidationall 14
IMMTinner ­membrane ­mitochondrial ­proteinHMP, MICOS60, MINOS2, Mic60, P87, P87/89, P89, PIG4, PIG52, Q16891, gene:1098930literature, targetP signal, yeast mito homolog, mito protein domain+, coexpression++, MS/MS++MIMMitochondrial dynamics and surveillance > Cristae formation > MICOS complexall 14
TIMM9translocase ­of ­inner ­mitochondrial ­membrane ­9TIM9, TIM9A, Q9Y5J7, gene:2652030literature, APEX_IMS, yeast mito homolog++, mito protein domain+, induction, coexpression+, MS/MS++MIMProtein import, sorting and homeostasis > Protein homeostasis > Chaperones;
Protein import, sorting and homeostasis > Protein import and sorting
all 14
SLC25A4solute ­carrier ­family ­25 ­member ­4AAC1, ANT, ANT 1, ANT1, MTDPS12, MTDPS12A, PEO2, PEO3, PEOA2, T1, P12235, gene:29130literature, yeast mito homolog++, mito protein domain+, induction, coexpression, MS/MS++MIMMetabolism > Nucleotide metabolism > Nucleotide import;
Signaling > Calcium homeostasis > Mitochondrial permeability transition pore;
Small molecule transport > SLC25A family
all 14
SAMM50SAMM50 ­sorting ­and ­assembly ­machinery ­componentCGI-51, OMP85, SAM50, TOB55, TRG-3, YNL026W, Q9Y512, gene:2581330literature, APEX_IMS, APEX_matrix, yeast mito homolog++, Rickettsial homolog, induction, coexpression++, MS/MS++MOMProtein import, sorting and homeostasis > Protein import and sorting > SAM;
Mitochondrial dynamics and surveillance > Intramitochondrial membrane interactions
all 14
NDUFS2NADH:ubiquinone ­oxidoreductase ­core ­subunit ­S2CI-49, MC1DN6, O75306, gene:472030literature, APEX_matrix, targetP signal, Rickettsial homolog, mito protein domain, induction, coexpression++, MS/MS++MIMOXPHOS > Complex I > CI subunits;
Metabolism > Metals and cofactors > Fe-S-containing proteins;
OXPHOS > OXPHOS subunits
all 14
NDUFV1NADH:ubiquinone ­oxidoreductase ­core ­subunit ­V1CI-51K, CI51KD, MC1DN4, UQOR1, P49821, gene:472330literature, APEX_matrix, targetP signal, Rickettsial homolog, mito protein domain, induction, coexpression++, MS/MS++MIMOXPHOS > Complex I > CI subunits;
Metabolism > Metals and cofactors > Fe-S-containing proteins;
OXPHOS > OXPHOS subunits
all 14
ACO2aconitase ­2ACONM, HEL-S-284, ICRD, OCA8, OPA9, Q99798, gene:5030literature, APEX_matrix, targetP signal, Rickettsial homolog, mito protein domain, induction, coexpression++, MS/MS++MatrixMetabolism > Carbohydrate metabolism > TCA cycle;
Metabolism > Metals and cofactors > Fe-S-containing proteins
all 14
SUPV3L1Suv3 ­like ­RNA ­helicaseSUV3, Q8IYB8, gene:683230literature, APEX_matrix, targetP signal+, yeast mito homolog++, mito protein domain, induction, coexpression+, MS/MS+MatrixMitochondrial central dogma > mtRNA metabolism > mtRNA granules;
Mitochondrial central dogma > mtRNA metabolism > mtRNA stability and decay
cerebrum, cerebellum, brainstem, spinalcord, kidney, liver, heart, skeletalmuscle, adipose, largeintestine, stomach, placenta, testis
FECHferrochelataseEPP, EPP1, FCE, P22830, gene:223530literature, APEX_matrix, targetP signal+, yeast mito homolog++, Rickettsial homolog, mito protein domain, coexpression, MS/MS++MIMMetabolism > Metals and cofactors > Heme synthesis and processing;
Metabolism > Metals and cofactors > Fe-S-containing proteins
all 14
MTIF2mitochondrial ­translational ­initiation ­factor ­2P46199, gene:452830literature, APEX_matrix, targetP signal, yeast mito homolog++, Rickettsial homolog, mito protein domain, induction, coexpression++, MS/MSMatrixMitochondrial central dogma > Translation > Translation factorsall 14
PHBprohibitinHEL-215, HEL-S-54e, PHB1, P35232, gene:524529literature, yeast mito homolog+, Rickettsial homolog, mito protein domain, induction, coexpression++, MS/MS++MIMProtein import, sorting and homeostasis > Protein homeostasis > Chaperonesall 14
HIBCH3-hydroxyisobutyryl-CoA ­hydrolaseHIBYLCOAH, Q6NVY1, gene:2627529literature, APEX_matrix, targetP signal+, yeast mito homolog+, mito protein domain, induction, coexpression, MS/MS++MatrixMetabolism > Amino acid metabolism > BCAA metabolismall 14
MRPS2mitochondrial ­ribosomal ­protein ­S2CGI-91, COXPD36, MRP-S2, S2mt, Q9Y399, gene:5111629literature, APEX_matrix, targetP signal, yeast mito homolog++, Rickettsial homolog, mito protein domain, induction, coexpression+, MS/MS+MatrixMitochondrial central dogma > Translation > Mitochondrial ribosomecerebrum, cerebellum, brainstem, spinalcord, liver, heart, adipose, smallintestine, largeintestine, stomach, placenta, testis
HSPA9heat ­shock ­protein ­family ­A ­(Hsp70) ­member ­9CRP40, CSA, EVPLS, GRP-75, GRP75, HEL-S-124m, HSPA9B, MOT, MOT2, MTHSP75, PBP74, SAAN, SIDBA4, P38646, gene:331329APEX_matrix, targetP signal+, yeast mito homolog, Rickettsial homolog, mito protein domain, induction, coexpression, MS/MS++MatrixMetabolism > Metals and cofactors > Fe-S cluster biosynthesisall 14
SURF1SURF1 ­cytochrome ­c ­oxidase ­assembly ­factorCMT4K, Q15526, gene:683429literature, targetP signal, yeast mito homolog++, Rickettsial homolog, mito protein domain+, coexpression, MS/MSMIMOXPHOS > Complex IV > CIV assembly factors;
OXPHOS > OXPHOS assembly factors
cerebrum, cerebellum, brainstem, spinalcord, kidney, liver, adipose, smallintestine, largeintestine, stomach, placenta, testis
PRDX3peroxiredoxin ­3AOP-1, AOP1, HBC189, MER5, PRO1748, SP-22, prx-III, P30048, gene:1093529literature, APEX_IMS, APEX_matrix, targetP signal+, Rickettsial homolog, mito protein domain, induction, coexpression++, MS/MS++MatrixMetabolism > Detoxification > ROS and glutathione metabolismall 14
GHITMgrowth ­hormone ­inducible ­transmembrane ­proteinDERP2, HSPC282, MICS1, My021, PTD010, TMBIM5, Q9H3K2, gene:2706929literature, APEX_IMS, targetP signal+, Rickettsial homolog, mito protein domain+, induction, coexpression++, MS/MSMIMMitochondrial dynamics and surveillance > Apoptosisall 14
GUF1GUF1 ­homolog, ­GTPaseEF-4, EF4, EIEE40, Q8N442, gene:6055829APEX_matrix, targetP signal, yeast mito homolog++, Rickettsial homolog, mito protein domain+, coexpression, MS/MS+MatrixMitochondrial central dogma > Translationcerebrum, cerebellum, brainstem, spinalcord, kidney, liver, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
TIMM13translocase ­of ­inner ­mitochondrial ­membrane ­13TIM13, TIM13B, TIMM13A, TIMM13B, ppv1, Q9Y5L4, gene:2651729literature, APEX_IMS, yeast mito homolog++, mito protein domain+, induction, coexpression, MS/MS++MIMProtein import, sorting and homeostasis > Protein homeostasis > Chaperones;
Protein import, sorting and homeostasis > Protein import and sorting
all 14
LYRM4LYR ­motif ­containing ­4C6orf149, CGI-203, COXPD19, ISD11, Q9HD34, gene:5712829literature, APEX_matrix, targetP signal+, yeast mito homolog, mito protein domain+, induction, coexpression, MS/MS++MatrixMetabolism > Metals and cofactors > Fe-S cluster biosynthesisall 14
MRPL16mitochondrial ­ribosomal ­protein ­L16L16mt, MRP-L16, PNAS-111, Q9NX20, gene:5494828literature, yeast mito homolog++, Rickettsial homolog, mito protein domain, coexpression++, MS/MS+MatrixMitochondrial central dogma > Translation > Mitochondrial ribosomecerebrum, cerebellum, brainstem, spinalcord, kidney, liver, heart, skeletalmuscle, adipose, smallintestine, largeintestine, placenta, testis
MRPL40mitochondrial ­ribosomal ­protein ­L40L40mt, MRP-L22, MRP-L40, MRPL22, NLVCF, URIM, Q9NQ50, gene:6497628literature, APEX_matrix, targetP signal+, yeast mito homolog++, mito protein domain+, induction, coexpression, MS/MSMatrixMitochondrial central dogma > Translation > Mitochondrial ribosomecerebrum, cerebellum, brainstem, spinalcord, kidney, liver, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
IDH3Gisocitrate ­dehydrogenase ­(NAD(+)) ­3 ­non-catalytic ­subunit ­gammaH-IDHG, P51553, gene:342128literature, APEX_matrix, yeast mito homolog+, Rickettsial homolog, mito protein domain, induction, coexpression++, MS/MS++MatrixMetabolism > Carbohydrate metabolism > TCA cycleall 14
SDHCsuccinate ­dehydrogenase ­complex ­subunit ­CCYB560, CYBL, PGL3, QPS1, SDH3, Q99643, gene:639128literature, targetP signal+, Rickettsial homolog, mito protein domain, induction, coexpression++, MS/MS+MIMOXPHOS > Complex II > CII subunits;
Metabolism > Carbohydrate metabolism > TCA cycle;
Metabolism > Metals and cofactors > Heme-containing proteins;
OXPHOS > OXPHOS subunits
all 14
NDUFB5NADH:ubiquinone ­oxidoreductase ­subunit ­B5CISGDH, SGDH, O43674, gene:471128literature, APEX_IMS, APEX_matrix, targetP signal+, mito protein domain+, induction, coexpression++, MS/MS+MIMOXPHOS > Complex I > CI subunits;
OXPHOS > OXPHOS subunits
all 14
SDHAF2succinate ­dehydrogenase ­complex ­assembly ­factor ­2C11orf79, PGL2, SDH5, Q9NX18, gene:5494928GFP, targetP signal, yeast mito homolog++, Rickettsial homolog, mito protein domain+, induction, coexpression, MS/MSMatrixOXPHOS > Complex II > CII assembly factors;
OXPHOS > OXPHOS assembly factors
cerebrum, cerebellum, brainstem, spinalcord, kidney, liver, heart, adipose, smallintestine, largeintestine, stomach, placenta, testis
COQ10Acoenzyme ­Q10AQ96MF6, gene:9305828targetP signal, yeast mito homolog+, Rickettsial homolog, mito protein domain+, induction, MS/MS++MIMMetabolism > Metals and cofactors > Coenzyme Q metabolismcerebrum, cerebellum, brainstem, spinalcord, kidney, liver, heart, skeletalmuscle, adipose, largeintestine, stomach
GATD3Aglutamine ­amidotransferase ­like ­class ­1 ­domain ­containing ­3AC21orf33, ES1, GATD3, GT335, HES1, KNPH, KNPI, P30042, gene:820928APEX_matrix, targetP signal+, mito protein domain, induction, coexpression++, MS/MS++Matrixnoneall 14
TXN2thioredoxin ­2COXPD29, MT-TRX, MTRX, TRX2, TXN, Q99757, gene:2582828literature, APEX_matrix, targetP signal+, Rickettsial homolog, mito protein domain, induction, coexpression++, MS/MS+MatrixMetabolism > Vitamin metabolism > Vitamin C metabolism;
Metabolism > Detoxification > ROS and glutathione metabolism
all 14
MRPS18Amitochondrial ­ribosomal ­protein ­S18AHumanS18b, MRP-S18-3, MRP-S18-a, MRPS18-3, S18bmt, S18mt-a, Q9NVS2, gene:5516828literature, APEX_matrix, targetP signal, Rickettsial homolog, mito protein domain, induction, coexpression++, MS/MS++MatrixMitochondrial central dogma > Translation > Mitochondrial ribosomecerebrum, brainstem, spinalcord, liver, heart, adipose, smallintestine, largeintestine, stomach, placenta, testis
COX6Ccytochrome ­c ­oxidase ­subunit ­6CP09669, gene:134528literature, APEX_IMS, APEX_matrix, targetP signal, mito protein domain+, induction, coexpression++, MS/MS++MIMOXPHOS > Complex IV > CIV subunits;
OXPHOS > OXPHOS subunits
all 14
NDUFB9NADH:ubiquinone ­oxidoreductase ­subunit ­B9B22, CI-B22, LYRM3, MC1DN24, UQOR22, Q9Y6M9, gene:471528literature, APEX_IMS, APEX_matrix, targetP signal, mito protein domain+, induction, coexpression++, MS/MS++MIMOXPHOS > Complex I > CI subunits;
OXPHOS > OXPHOS subunits
all 14
MTCH2mitochondrial ­carrier ­2HSPC032, MIMP, SLC25A50, Q9Y6C9, gene:2378828literature, APEX_IMS, yeast mito homolog+, mito protein domain+, induction, coexpression+, MS/MS++MOMSmall molecule transport > SLC25A family;
Mitochondrial dynamics and surveillance > Fusion
all 14
NDUFA6NADH:ubiquinone ­oxidoreductase ­subunit ­A6B14, CI-B14, LYRM6, MC1DN33, NADHB14, P56556, gene:470028literature, APEX_matrix, mito protein domain+, induction, coexpression++, MS/MS++MIMOXPHOS > Complex I > CI subunits;
OXPHOS > OXPHOS subunits
all 14
SLC25A20solute ­carrier ­family ­25 ­member ­20CAC, CACT, O43772, gene:78828literature, yeast mito homolog+, mito protein domain+, induction, coexpression, MS/MS++MIMMetabolism > Lipid metabolism > Fatty acid oxidation;
Metabolism > Metals and cofactors > Carnitine synthesis and transport;
Metabolism > Metals and cofactors > Carnitine shuttle;
Small molecule transport > SLC25A family
all 14
MRPL1mitochondrial ­ribosomal ­protein ­L1BM022, L1MT, MRP-L1, Q9BYD6, gene:6500828literature, APEX_matrix, targetP signal, yeast mito homolog++, mito protein domain, induction, coexpression, MS/MS++MatrixMitochondrial central dogma > Translation > Mitochondrial ribosomecerebrum, cerebellum, spinalcord, liver, heart, adipose, smallintestine, largeintestine, stomach, placenta, testis
TIMM44translocase ­of ­inner ­mitochondrial ­membrane ­44TIM44, O43615, gene:1046928literature, APEX_matrix, targetP signal, yeast mito homolog, mito protein domain, induction, coexpression++, MS/MS++MIMProtein import, sorting and homeostasis > Protein import and sorting > Import motorall 14
COX17cytochrome ­c ­oxidase ­copper ­chaperone ­COX17Q14061, gene:1006328literature, APEX_IMS, yeast mito homolog, mito protein domain+, induction, coexpression++, MS/MS++IMSOXPHOS > Complex IV > CIV assembly factors;
Metabolism > Metals and cofactors > Copper metabolism;
OXPHOS > OXPHOS assembly factors
heart, adipose, smallintestine, placenta, testis
MICOS10mitochondrial ­contact ­site ­and ­cristae ­organizing ­system ­subunit ­10C1orf151, MINOS1, MIO10, Mic10, Q5TGZ0, gene:44057428literature, APEX_IMS, yeast mito homolog, mito protein domain+, induction, coexpression++, MS/MS++MIMMitochondrial dynamics and surveillance > Cristae formation > MICOS complexall 14
SOD2superoxide ­dismutase ­2GClnc1, IPO-B, IPOB, MNSOD, MVCD6, Mn-SOD, P04179, gene:664828literature, APEX_matrix, targetP signal, yeast mito homolog++, Rickettsial homolog, mito protein domain, induction, MS/MS++MatrixMetabolism > Detoxification > ROS and glutathione metabolismall 14
COX6B1cytochrome ­c ­oxidase ­subunit ­6B1COX6B, COXG, COXVIb1, P14854, gene:134028literature, APEX_IMS, yeast mito homolog+, mito protein domain+, coexpression++, MS/MS++MIMOXPHOS > Complex IV > CIV subunits;
OXPHOS > OXPHOS subunits
all 14
VDAC1voltage ­dependent ­anion ­channel ­1PORIN, VDAC-1, P21796, gene:741628literature, APEX_IMS, yeast mito homolog+, mito protein domain+, coexpression++, MS/MS++MOMSignaling > Calcium homeostasis > Mitochondrial permeability transition pore;
Mitochondrial dynamics and surveillance > Organelle contact sites;
Small molecule transport
all 14
CLPPcaseinolytic ­mitochondrial ­matrix ­peptidase ­proteolytic ­subunitDFNB81, PRLTS3, Q16740, gene:819228literature, APEX_matrix, targetP signal+, Rickettsial homolog, mito protein domain, induction, coexpression+, MS/MS++MatrixProtein import, sorting and homeostasis > Protein homeostasis > Proteasesall 14
HADHhydroxyacyl-CoA ­dehydrogenaseHAD, HADH1, HADHSC, HCDH, HHF4, MSCHAD, SCHAD, Q16836, gene:303328literature, APEX_matrix, targetP signal, Rickettsial homolog, mito protein domain, induction, coexpression++, MS/MS++MatrixMetabolism > Lipid metabolism > Fatty acid oxidation;
Metabolism > Amino acid metabolism > Lysine metabolism
all 14
ACADLacyl-CoA ­dehydrogenase ­long ­chainACAD4, LCAD, P28330, gene:3328literature, targetP signal+, mito protein domain+, induction, coexpression+, MS/MS++MIMMetabolism > Lipid metabolism > Fatty acid oxidationall 14
ACAT1acetyl-CoA ­acetyltransferase ­1ACAT, MAT, T2, THIL, P24752, gene:3828literature, APEX_matrix, targetP signal+, Rickettsial homolog, mito protein domain, induction, coexpression+, MS/MS++MatrixMetabolism > Carbohydrate metabolism > Ketone metabolism;
Metabolism > Lipid metabolism > Fatty acid oxidation;
Metabolism > Amino acid metabolism > BCAA metabolism;
Metabolism > Amino acid metabolism > Lysine metabolism
all 14
MALSU1mitochondrial ­assembly ­of ­ribosomal ­large ­subunit ­1C7orf30, mtRsfA, Q96EH3, gene:11541628literature, APEX_matrix, targetP signal+, yeast mito homolog++, Rickettsial homolog, mito protein domain, coexpression+, MS/MSMatrixMitochondrial central dogma > Translation > Mitochondrial ribosome assemblycerebrum, cerebellum, liver, smallintestine, largeintestine, placenta
CLPXcaseinolytic ­mitochondrial ­matrix ­peptidase ­chaperone ­subunit ­XEPP2, O76031, gene:1084527literature, APEX_matrix, yeast mito homolog++, Rickettsial homolog, mito protein domain, induction, coexpression, MS/MS++MatrixProtein import, sorting and homeostasis > Protein homeostasis > Proteasesall 14
NDUFS4NADH:ubiquinone ­oxidoreductase ­subunit ­S4AQDQ, CI-18, CI-18 kDa, CI-AQDQ, MC1DN1, O43181, gene:472427literature, APEX_matrix, targetP signal+, mito protein domain+, coexpression++, MS/MS++MIMOXPHOS > Complex I > CI subunits;
OXPHOS > OXPHOS subunits
all 14
MRPL4mitochondrial ­ribosomal ­protein ­L4CGI-28, L4mt, Q9BYD3, gene:5107327literature, APEX_matrix, targetP signal, yeast mito homolog++, Rickettsial homolog, mito protein domain, induction, MS/MS++MatrixMitochondrial central dogma > Translation > Mitochondrial ribosomecerebrum, brainstem, spinalcord, kidney, liver, adipose, smallintestine, largeintestine, placenta, testis
C1QBPcomplement ­C1q ­binding ­proteinCOXPD33, GC1QBP, HABP1, SF2AP32, SF2p32, gC1Q-R, gC1qR, p32, Q07021, gene:70827literature, APEX_matrix, targetP signal+, yeast mito homolog++, mito protein domain, induction, coexpression, MS/MS++MatrixSignaling > Immune responseall 14
PITRM1pitrilysin ­metallopeptidase ­1MP1, PreP, Q5JRX3, gene:1053127literature, APEX_matrix, targetP signal, yeast mito homolog++, mito protein domain+, coexpression, MS/MS++MatrixProtein import, sorting and homeostasis > Protein homeostasis > Proteasesall 14
UQCC1ubiquinol-cytochrome ­c ­reductase ­complex ­assembly ­factor ­1BFZB, C20orf44, CBP3, UQCC, Q9NVA1, gene:5524527literature, APEX_matrix, targetP signal, yeast mito homolog, mito protein domain+, coexpression++, MS/MS++MIMOXPHOS > Complex III > CIII assembly factors;
OXPHOS > OXPHOS assembly factors
cerebrum, cerebellum, brainstem, spinalcord, liver, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
MRPL55mitochondrial ­ribosomal ­protein ­L55AAVG5835, L55nt, MRP-L55, PRO19675, Q7Z7F7, gene:12830827literature, APEX_matrix, targetP signal, mito protein domain+, induction, coexpression++, MS/MS+MatrixMitochondrial central dogma > Translation > Mitochondrial ribosomecerebrum, cerebellum, brainstem, spinalcord, kidney, liver, heart, adipose, smallintestine, largeintestine, stomach, placenta, testis
MRM1mitochondrial ­rRNA ­methyltransferase ­1Q6IN84, gene:7992227APEX_matrix, targetP signal+, yeast mito homolog++, Rickettsial homolog, mito protein domain+, induction, coexpressionMatrixMitochondrial central dogma > mtRNA metabolism > mtRNA granules;
Mitochondrial central dogma > mtRNA metabolism > mt-rRNA modifications
spinalcord, placenta, testis
MECRmitochondrial ­trans-2-enoyl-CoA ­reductaseCGI-63, DYTOABG, ETR1, FASN2B, NRBF1, Q9BV79, gene:5110227literature, APEX_matrix, targetP signal+, yeast mito homolog++, mito protein domain, coexpression, MS/MS++MatrixMetabolism > Lipid metabolism > Type II fatty acid synthesisall 14
MRPL44mitochondrial ­ribosomal ­protein ­L44COXPD16, L44MT, MRP-L44, Q9H9J2, gene:6508027literature, APEX_matrix, targetP signal, yeast mito homolog++, induction, coexpression+, MS/MS++MatrixMitochondrial central dogma > Translation > Mitochondrial ribosomecerebrum, spinalcord, liver, heart, adipose, smallintestine, largeintestine, stomach, placenta, testis
HSDL2hydroxysteroid ­dehydrogenase ­like ­2C9orf99, SDR13C1, Q6YN16, gene:8426327APEX_matrix, GFP, Rickettsial homolog, mito protein domain+, induction, coexpression+, MS/MS++Matrixnoneall 14
MRPS14mitochondrial ­ribosomal ­protein ­S14COXPD38, DJ262D12.2, HSMRPS14, MRP-S14, S14mt, O60783, gene:6393127literature, APEX_matrix, targetP signal, yeast mito homolog++, Rickettsial homolog, mito protein domain, coexpression, MS/MS++MatrixMitochondrial central dogma > Translation > Mitochondrial ribosomecerebrum, cerebellum, brainstem, spinalcord, kidney, liver, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
ABCB8ATP ­binding ­cassette ­subfamily ­B ­member ­8EST328128, M-ABC1, MABC1, MITOSUR, Q9NUT2, gene:1119427literature, targetP signal+, Rickettsial homolog, mito protein domain, coexpression++, MS/MS++MIMSmall molecule transport > ABC transportersall 14
ATPAF2ATP ­synthase ­mitochondrial ­F1 ­complex ­assembly ­factor ­2ATP12, ATP12p, LP3663, MC5DN1, Q8N5M1, gene:9164727literature, targetP signal, yeast mito homolog++, mito protein domain+, induction, coexpression, MS/MSMatrixOXPHOS > Complex V > CV assembly factors;
OXPHOS > OXPHOS assembly factors
cerebrum, cerebellum, brainstem, spinalcord, liver, heart, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
NDUFA9NADH:ubiquinone ­oxidoreductase ­subunit ­A9CC6, CI-39k, CI39k, COQ11, MC1DN26, NDUFS2L, SDR22E1, Q16795, gene:470427literature, APEX_matrix, targetP signal+, mito protein domain, induction, coexpression++, MS/MS++MIMOXPHOS > Complex I > CI subunits;
OXPHOS > OXPHOS subunits
all 14
COA6cytochrome ­c ­oxidase ­assembly ­factor ­6C1orf31, CEMCOX4, Q5JTJ3, gene:38875327APEX_IMS, yeast mito homolog++, mito protein domain+, induction, coexpression+, MS/MSIMSOXPHOS > Complex IV > CIV assembly factors;
OXPHOS > OXPHOS assembly factors
cerebrum, cerebellum, spinalcord, liver, adipose, smallintestine, largeintestine, placenta, testis
POLRMTRNA ­polymerase ­mitochondrialAPOLMT, MTRNAP, MTRPOL, h-mtRPOL, O00411, gene:544227literature, APEX_matrix, targetP signal, yeast mito homolog++, mito protein domain+, coexpression++, MS/MSMatrixMitochondrial central dogma > mtDNA maintenance > mtDNA replication;
Mitochondrial central dogma > mtDNA maintenance > mtDNA nucleoid;
Mitochondrial central dogma > mtRNA metabolism > Transcription
cerebrum, cerebellum, brainstem, spinalcord, kidney, skeletalmuscle, adipose, largeintestine, stomach, placenta, testis
ISCUiron-sulfur ­cluster ­assembly ­enzyme2310020H20Rik, HML, ISU2, NIFU, NIFUN, hnifU, Q9H1K1, gene:2347927literature, APEX_matrix, targetP signal+, yeast mito homolog++, Rickettsial homolog, mito protein domain, MS/MS++MatrixMetabolism > Metals and cofactors > Fe-S cluster biosynthesis;
Metabolism > Metals and cofactors > Fe-S-containing proteins
all 14
RTN4IP1reticulon ­4 ­interacting ­protein ­1NIMP, OPA10, Q8WWV3, gene:8481627literature, APEX_matrix, targetP signal, yeast mito homolog, mito protein domain, induction, coexpression++, MS/MS++Matrixnoneall 14
OGDHLoxoglutarate ­dehydrogenase ­LQ9ULD0, gene:5575327APEX_matrix, targetP signal+, yeast mito homolog+, Rickettsial homolog, mito protein domain+, MS/MS++MatrixMetabolism > Carbohydrate metabolism > TCA cycleall 14
ATP5F1CATP ­synthase ­F1 ­subunit ­gammaATP5C, ATP5C1, ATP5CL1, P36542, gene:50927literature, yeast mito homolog++, Rickettsial homolog, mito protein domain, induction, MS/MS++MIMOXPHOS > Complex V > CV subunits;
OXPHOS > OXPHOS subunits
all 14
BCKDKbranched ­chain ­keto ­acid ­dehydrogenase ­kinaseBCKDKD, BDK, O14874, gene:1029527literature, APEX_matrix, targetP signal, yeast mito homolog+, Rickettsial homolog, mito protein domain+, MS/MS++MatrixMetabolism > Amino acid metabolism > BCAA metabolism;
Metabolism > Amino acid metabolism > BCAA dehydrogenase complex
cerebrum, cerebellum, spinalcord, kidney, liver, heart, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
GFM2GTP ­dependent ­ribosome ­recycling ­factor ­mitochondrial ­2EF-G2mt, EFG2, MRRF2, MST027, MSTP027, RRF, RRF2, RRF2mt, hEFG2, mEF-G 2, Q969S9, gene:8434027literature, APEX_matrix, yeast mito homolog++, Rickettsial homolog, mito protein domain, coexpression++, MS/MS++MatrixMitochondrial central dogma > Translation > Translation factorsall 14
NDUFAF4NADH:ubiquinone ­oxidoreductase ­complex ­assembly ­factor ­4C6orf66, HRPAP20, HSPC125, MC1DN15, My013, bA22L21.1, Q9P032, gene:2907827APEX_matrix, GFP, mito protein domain+, induction, coexpression++, MS/MS++MIMOXPHOS > Complex I > CI assembly factors;
OXPHOS > OXPHOS assembly factors
all 14
SLC25A35solute ­carrier ­family ­25 ­member ­35Q3KQZ1, gene:39951227APEX_matrix, yeast mito homolog+, mito protein domain+, induction, coexpression, MS/MS++MIMSmall molecule transport > SLC25A familycerebrum, cerebellum, kidney, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
SLC25A11solute ­carrier ­family ­25 ­member ­11OGC, PGL6, SLC20A4, Q02978, gene:840227literature, yeast mito homolog+, mito protein domain+, induction, coexpression, MS/MS++MIMMetabolism > Carbohydrate metabolism > Gluconeogenesis;
Metabolism > Carbohydrate metabolism > Malate-aspartate shuttle;
Metabolism > Carbohydrate metabolism > TCA-associated;
Small molecule transport > SLC25A family
all 14
TIMM8Btranslocase ­of ­inner ­mitochondrial ­membrane ­8 ­homolog ­BDDP2, TIM8B, Q9Y5J9, gene:2652127literature, yeast mito homolog+, mito protein domain+, induction, coexpression, MS/MS++MIMProtein import, sorting and homeostasis > Protein homeostasis > Chaperones;
Protein import, sorting and homeostasis > Protein import and sorting
cerebrum, cerebellum, brainstem, spinalcord, kidney, liver, heart, adipose, smallintestine, largeintestine, stomach, placenta, testis
MCATmalonyl-CoA-acyl ­carrier ­protein ­transacylaseFASN2C, MCT, MCT1, MT, NET62, fabD, Q8IVS2, gene:2734926literature, APEX_matrix, targetP signal, yeast mito homolog++, Rickettsial homolog, mito protein domain, coexpression, MS/MS++MatrixMetabolism > Lipid metabolism > Type II fatty acid synthesisspinalcord, kidney, liver, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
IBA57iron-sulfur ­cluster ­assembly ­factor ­IBA57C1orf69, MMDS3, SPG74, Q5T440, gene:20020526APEX_matrix, targetP signal, yeast mito homolog++, Rickettsial homolog, mito protein domain, coexpression, MS/MS++MatrixMetabolism > Metals and cofactors > Fe-S cluster biosynthesiscerebrum, cerebellum, brainstem, spinalcord, kidney, liver, heart, adipose, smallintestine, largeintestine, stomach, placenta, testis
PHB2prohibitin ­2BAP, BCAP37, Bap37, PNAS-141, REA, hBAP, p22, Q99623, gene:1133126literature, yeast mito homolog++, Rickettsial homolog, mito protein domain, induction, coexpression, MS/MS++MIMProtein import, sorting and homeostasis > Protein homeostasis > Chaperonesall 14
DAP3death ­associated ­protein ­3DAP-3, MRP-S29, MRPS29, S29mt, bMRP-10, P51398, gene:781826literature, APEX_matrix, targetP signal, yeast mito homolog++, mito protein domain+, coexpression, MS/MS++MatrixMitochondrial central dogma > Translation > Mitochondrial ribosomeall 14
CMC2C-X9-C ­motif ­containing ­22310061C15Rik, C16orf61, DC13, Q9NRP2, gene:5694226APEX_IMS, yeast mito homolog++, mito protein domain+, induction, coexpression, MS/MS+IMSOXPHOS > Complex IV > CIV assembly factors;
OXPHOS > OXPHOS assembly factors
cerebrum, cerebellum, spinalcord, liver, heart, adipose, largeintestine, stomach, placenta, testis
SDHAF1succinate ­dehydrogenase ­complex ­assembly ­factor ­1LYRM8, A6NFY7, gene:64409626literature, targetP signal+, yeast mito homolog, mito protein domain+, induction, coexpression+, MS/MSMatrixOXPHOS > Complex II > CII assembly factors;
OXPHOS > OXPHOS assembly factors
cerebrum, cerebellum, brainstem, spinalcord, kidney, heart, skeletalmuscle, adipose, largeintestine, stomach, placenta, testis
MRPS17mitochondrial ­ribosomal ­protein ­S17HSPC011, MRP-S17, RPMS17, S17mt, Q9Y2R5, gene:5137326literature, targetP signal, yeast mito homolog++, Rickettsial homolog, mito protein domain, induction, coexpression, MS/MS+MatrixMitochondrial central dogma > Translation > Mitochondrial ribosomeall 14
ADHFE1alcohol ­dehydrogenase ­iron ­containing ­1ADH8, HMFT2263, HOT, Q8IWW8, gene:13787226targetP signal+, yeast mito homolog++, mito protein domain, coexpression, MS/MS++MatrixMetabolism > Amino acid metabolism > GABA metabolismkidney, liver, heart, adipose, stomach, placenta
NFS1NFS1 ­cysteine ­desulfuraseHUSSY-08, IscS, NIFS, Q9Y697, gene:905426literature, APEX_matrix, targetP signal, Rickettsial homolog, mito protein domain+, coexpression+, MS/MS++MatrixMetabolism > Metals and cofactors > Fe-S cluster biosynthesis;
Metabolism > Metals and cofactors > Fe-S-containing proteins
all 14
NDUFAF5NADH:ubiquinone ­oxidoreductase ­complex ­assembly ­factor ­5C20orf7, MC1DN16, bA526K24.2, dJ842G6.1, Q5TEU4, gene:7913326literature, APEX_matrix, GFP, targetP signal+, Rickettsial homolog, mito protein domain+, induction, coexpression, MS/MSMIMOXPHOS > Complex I > CI assembly factors;
OXPHOS > OXPHOS assembly factors
cerebellum, brainstem, skeletalmuscle, largeintestine, stomach, placenta
MRPL17mitochondrial ­ribosomal ­protein ­L17L17mt, LIP2, MRP-L17, MRP-L26, RPL17L, RPML26, Q9NRX2, gene:6387526literature, APEX_matrix, targetP signal+, yeast mito homolog++, Rickettsial homolog, mito protein domain, coexpression, MS/MSMatrixMitochondrial central dogma > Translation > Mitochondrial ribosomeall 14
TACO1translational ­activator ­of ­cytochrome ­c ­oxidase ­ICCDC44, Q9BSH4, gene:5120426literature, APEX_matrix, targetP signal+, yeast mito homolog, Rickettsial homolog, mito protein domain+, MS/MS++MatrixMitochondrial central dogma > Translation > Translation factors;
OXPHOS > Complex IV > CIV assembly factors;
OXPHOS > OXPHOS assembly factors
all 14
MFN1mitofusin ­1hfzo1, hfzo2, Q8IWA4, gene:5566926literature, yeast mito homolog++, mito protein domain+, induction, coexpression, MS/MS+MOMMitochondrial dynamics and surveillance > Fusion;
Mitochondrial dynamics and surveillance > Organelle contact sites
cerebellum, brainstem, spinalcord, kidney, skeletalmuscle, adipose, largeintestine, stomach, placenta, testis
PPTC7protein ­phosphatase ­targeting ­COQ7TA-PP2C, TAPP2C, Q8NI37, gene:16076026APEX_matrix, yeast mito homolog, mito protein domain+, induction, coexpression, MS/MS++MatrixSignalingcerebrum, cerebellum, brainstem, spinalcord, heart, skeletalmuscle, largeintestine, placenta, testis
MRPL11mitochondrial ­ribosomal ­protein ­L11CGI-113, L11MT, MRP-L11, Q9Y3B7, gene:6500326literature, targetP signal, yeast mito homolog++, Rickettsial homolog, mito protein domain, coexpression+, MS/MS+MatrixMitochondrial central dogma > Translation > Mitochondrial ribosomeall 14
COX4I1cytochrome ­c ­oxidase ­subunit ­4I1COX IV-1, COX4, COX4-1, COXIV, COXIV-1, P13073, gene:132726literature, APEX_IMS, APEX_matrix, targetP signal, mito protein domain+, coexpression++, MS/MS++MIMOXPHOS > Complex IV > CIV subunits;
OXPHOS > OXPHOS subunits
all 14
NDUFS6NADH:ubiquinone ­oxidoreductase ­subunit ­S6CI-13kA, CI-13kD-A, CI13KDA, MC1DN9, O75380, gene:472626literature, APEX_matrix, targetP signal, mito protein domain+, coexpression++, MS/MS++MIMOXPHOS > Complex I > CI subunits;
OXPHOS > OXPHOS subunits
all 14
MRPL13mitochondrial ­ribosomal ­protein ­L13L13, L13A, L13mt, RPL13, RPML13, Q9BYD1, gene:2899826literature, APEX_matrix, targetP signal, yeast mito homolog, Rickettsial homolog, mito protein domain, induction, coexpression, MS/MS++MatrixMitochondrial central dogma > Translation > Mitochondrial ribosomeall 14
OXCT13-oxoacid ­CoA-transferase ­1OXCT, SCOT, P55809, gene:501926literature, APEX_matrix, targetP signal, mito protein domain, induction, coexpression++, MS/MS++MatrixMetabolism > Carbohydrate metabolism > Ketone metabolismcerebrum, cerebellum, brainstem, spinalcord, kidney, heart, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
COX5Bcytochrome ­c ­oxidase ­subunit ­5BCOXVB, P10606, gene:132926literature, APEX_IMS, APEX_matrix, targetP signal+, yeast mito homolog++, mito protein domain+, MS/MS++MIMOXPHOS > Complex IV > CIV subunits;
OXPHOS > OXPHOS subunits
all 14
PDK2pyruvate ­dehydrogenase ­kinase ­2PDHK2, PDKII, Q15119, gene:516426literature, APEX_matrix, yeast mito homolog+, mito protein domain+, induction, coexpression, MS/MS++MatrixMetabolism > Carbohydrate metabolism > Pyruvate metabolismall 14
ALAS15'-aminolevulinate ­synthase ­1ALAS, ALAS-H, ALAS3, ALASH, MIG4, P13196, gene:21126literature, APEX_matrix, targetP signal, yeast mito homolog+, Rickettsial homolog, mito protein domain+, induction, coexpression, MS/MSMatrixMetabolism > Metals and cofactors > Heme synthesis and processingliver, heart, adipose, largeintestine, placenta
MRPL33mitochondrial ­ribosomal ­protein ­L33C2orf1, L33mt, MRP-L33, RPL33L, O75394, gene:955326literature, yeast mito homolog++, Rickettsial homolog, mito protein domain, coexpression, MS/MS++MatrixMitochondrial central dogma > Translation > Mitochondrial ribosomecerebrum, cerebellum, brainstem, spinalcord, kidney, liver, adipose, smallintestine, stomach, placenta, testis
MTO1mitochondrial ­tRNA ­translation ­optimization ­1CGI-02, COXPD10, Q9Y2Z2, gene:2582126literature, APEX_matrix, targetP signal, yeast mito homolog++, Rickettsial homolog, mito protein domain, coexpression++MatrixMitochondrial central dogma > mtRNA metabolism > mt-tRNA modifications;
Metabolism > Vitamin metabolism > Folate and 1-C metabolism
cerebellum, spinalcord, stomach, placenta
LIASlipoic ­acid ­synthetaseHGCLAS, HUSSY-01, LAS, LIP1, LS, PDHLD, O43766, gene:1101926literature, APEX_matrix, targetP signal, yeast mito homolog, Rickettsial homolog, mito protein domain, induction, coexpression++, MS/MSMatrixMetabolism > Lipid metabolism > Lipoate insertion;
Metabolism > Metals and cofactors > Fe-S-containing proteins
brainstem, skeletalmuscle, adipose, largeintestine, placenta
NDUFA5NADH:ubiquinone ­oxidoreductase ­subunit ­A5B13, CI-13KD-B, CI-13kB, NUFM, UQOR13, Q16718, gene:469826literature, APEX_matrix, mito protein domain+, induction, coexpression++, MS/MS++MIMOXPHOS > Complex I > CI subunits;
OXPHOS > OXPHOS subunits
all 14
NDUFB6NADH:ubiquinone ­oxidoreductase ­subunit ­B6B17, CI, O95139, gene:471226literature, APEX_IMS, APEX_matrix, mito protein domain+, induction, coexpression++, MS/MS++MIMOXPHOS > Complex I > CI subunits;
OXPHOS > OXPHOS subunits
all 14
MTX2metaxin ­2metaxin-2, O75431, gene:1065126literature, mito protein domain+, induction, coexpression++, MS/MS++MOMProtein import, sorting and homeostasis > Protein import and sorting > SAM;
Mitochondrial dynamics and surveillance > Intramitochondrial membrane interactions
all 14
SUCLG2succinate-CoA ­ligase ­GDP-forming ­subunit ­betaG-SCS, GBETA, GTPSCS, Q96I99, gene:880126literature, APEX_matrix, targetP signal, yeast mito homolog+, Rickettsial homolog, mito protein domain, coexpression, MS/MS++MatrixMetabolism > Carbohydrate metabolism > TCA cycle;
Metabolism > Carbohydrate metabolism > Itaconate metabolism;
Metabolism > Nucleotide metabolism > Nucleotide synthesis and processing
all 14
FDX1ferredoxin ­1ADX, FDX, LOH11CR1D, P10109, gene:223026literature, APEX_matrix, targetP signal, yeast mito homolog+, Rickettsial homolog, mito protein domain, induction, coexpression, MS/MS+MatrixMetabolism > Lipid metabolism > Cholesterol, bile acid, steroid synthesis;
Metabolism > Metals and cofactors > Heme synthesis and processing;
Metabolism > Metals and cofactors > Fe-S cluster biosynthesis;
Metabolism > Metals and cofactors > Fe-S-containing proteins;
Metabolism > Vitamin metabolism > Vitamin D metabolism
all 14
SLC25A1solute ­carrier ­family ­25 ­member ­1CMS23, CTP, D2L2AD, SEA, SLC20A3, P53007, gene:657626literature, yeast mito homolog++, mito protein domain+, coexpression, MS/MS++MIMMetabolism > Carbohydrate metabolism > Gluconeogenesis;
Metabolism > Carbohydrate metabolism > TCA-associated;
Metabolism > Lipid metabolism;
Small molecule transport > SLC25A family
cerebrum, cerebellum, brainstem, spinalcord, kidney, liver, adipose, smallintestine, largeintestine, stomach, placenta, testis
MRPS28mitochondrial ­ribosomal ­protein ­S28COXPD47, HSPC007, MRP-S28, MRP-S35, MRPS35, Q9Y2Q9, gene:2895726literature, targetP signal, mito protein domain+, coexpression++, MS/MS++MatrixMitochondrial central dogma > Translation > Mitochondrial ribosomeall 14
TIMM50translocase ­of ­inner ­mitochondrial ­membrane ­50MGCA9, TIM50, TIM50L, Q3ZCQ8, gene:9260926literature, APEX_IMS, yeast mito homolog++, mito protein domain, coexpression++, MS/MS++MIMProtein import, sorting and homeostasis > Protein import and sorting > TIM23 presequence pathwayall 14
VDAC2voltage ­dependent ­anion ­channel ­2POR, P45880, gene:741726literature, APEX_IMS, yeast mito homolog+, mito protein domain+, induction, coexpression, MS/MS++MOMSmall molecule transportall 14
SLC25A42solute ­carrier ­family ­25 ­member ­42MECREN, Q86VD7, gene:28443926literature, yeast mito homolog+, mito protein domain+, induction, coexpression, MS/MS++MIMMetabolism > Metals and cofactors > Coenzyme A metabolism;
Small molecule transport > SLC25A family
all 14
TOMM7translocase ­of ­outer ­mitochondrial ­membrane ­7TOM7, Q9P0U1, gene:5454326literature, yeast mito homolog++, mito protein domain+, coexpression, MS/MS++MOMProtein import, sorting and homeostasis > Protein import and sorting > TOM;
Mitochondrial dynamics and surveillance > Mitophagy;
Mitochondrial dynamics and surveillance > Autophagy
cerebrum, cerebellum, spinalcord, kidney, liver, skeletalmuscle, adipose, smallintestine, stomach, placenta, testis
ECH1enoyl-CoA ­hydratase ­1HPXEL, Q13011, gene:189126APEX_matrix, targetP signal+, yeast mito homolog+, mito protein domain, coexpression+, MS/MS++MatrixMetabolism > Lipid metabolismall 14
GATBglutamyl-tRNA ­amidotransferase ­subunit ­BCOXPD41, HSPC199, PET112, PET112L, O75879, gene:518825literature, APEX_matrix, targetP signal, yeast mito homolog, Rickettsial homolog, mito protein domain+, coexpression+, MS/MSMatrixMitochondrial central dogma > Translation > mt-tRNA synthetasescerebrum, skeletalmuscle, adipose, stomach, testis
BCS1LBCS1 ­homolog, ­ubiquinol-cytochrome ­c ­reductase ­complex ­chaperoneBCS, BCS1, BJS, FLNMS, GRACILE, Hs.6719, MC3DN1, PTD, h-BCS, h-BCS1, Q9Y276, gene:61725literature, APEX_matrix, yeast mito homolog++, Rickettsial homolog, mito protein domain+, coexpression+, MS/MS+MIMOXPHOS > Complex III > CIII assembly factors;
OXPHOS > OXPHOS assembly factors
cerebrum, cerebellum, brainstem, spinalcord, liver, heart, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
ERAL1Era ­like ­12S ­mitochondrial ­rRNA ­chaperone ­1CEGA, ERA, ERA-W, ERAL1A, ERAL1B, H-ERA, HERA-A, HERA-B, PRLTS6, O75616, gene:2628425APEX_matrix, targetP signal+, yeast mito homolog+, Rickettsial homolog, mito protein domain, induction, coexpression++MatrixMitochondrial central dogma > mtRNA metabolism > mtRNA granules;
Mitochondrial central dogma > Translation > Mitochondrial ribosome assembly
none
CMC1C-X9-C ­motif ­containing ­1C3orf68, Q7Z7K0, gene:15210025APEX_IMS, GFP, yeast mito homolog++, mito protein domain+, induction, coexpression, MS/MS+IMSOXPHOS > Complex IV > CIV assembly factors;
OXPHOS > OXPHOS assembly factors
cerebrum, cerebellum, brainstem, spinalcord, kidney, liver, adipose, smallintestine, largeintestine, stomach, placenta, testis
MRPL28mitochondrial ­ribosomal ­protein ­L28MAAT1, p15, Q13084, gene:1057325literature, APEX_matrix, yeast mito homolog++, Rickettsial homolog, coexpression, MS/MS++MatrixMitochondrial central dogma > Translation > Mitochondrial ribosomecerebrum, cerebellum, brainstem, spinalcord, liver, heart, adipose, smallintestine, largeintestine, stomach, placenta, testis
TSFMTs ­translation ­elongation ­factor, ­mitochondrialEFTS, EFTSMT, P43897, gene:1010225literature, APEX_matrix, targetP signal+, Rickettsial homolog, mito protein domain, induction, coexpression, MS/MS++MatrixMitochondrial central dogma > Translation > Translation factorscerebrum, cerebellum, brainstem, spinalcord, liver, heart, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
FXNfrataxinCyaY, FA, FARR, FRDA, X25, Q16595, gene:239525literature, APEX_matrix, targetP signal, yeast mito homolog++, Rickettsial homolog, mito protein domain, coexpression, MS/MS+MatrixMetabolism > Metals and cofactors > Fe-S cluster biosynthesisall 14
NFU1NFU1 ­iron-sulfur ­cluster ­scaffoldCGI-33, HIRIP, HIRIP5, MMDS1, NIFUC, Nfu, NifU, Q9UMS0, gene:2724725APEX_matrix, targetP signal, yeast mito homolog++, Rickettsial homolog, mito protein domain, coexpression, MS/MS+MatrixMetabolism > Metals and cofactors > Fe-S cluster biosynthesis;
Metabolism > Metals and cofactors > Fe-S-containing proteins
all 14
YARS2tyrosyl-tRNA ­synthetase ­2CGI-04, MLASA2, MT-TYRRS, TYRRS, Q9Y2Z4, gene:5106725literature, APEX_matrix, targetP signal, yeast mito homolog++, Rickettsial homolog, mito protein domain, coexpression, MS/MS+MatrixMitochondrial central dogma > Translation > mt-tRNA synthetasesbrainstem, spinalcord, kidney, adipose, smallintestine, largeintestine, stomach, placenta, testis
MRPL21mitochondrial ­ribosomal ­protein ­L21L21mt, MRP-L21, Q7Z2W9, gene:21992725literature, APEX_matrix, targetP signal, yeast mito homolog++, Rickettsial homolog, mito protein domain, coexpression, MS/MS+MatrixMitochondrial central dogma > Translation > Mitochondrial ribosomeall 14
ALDH4A1aldehyde ­dehydrogenase ­4 ­family ­member ­A1ALDH4, P5CD, P5CDh, P30038, gene:865925literature, APEX_matrix, targetP signal+, yeast mito homolog++, mito protein domain, coexpression, MS/MS++MatrixMetabolism > Amino acid metabolism > Proline metabolism;
Metabolism > Amino acid metabolism > Glyoxylate metabolism
all 14
TOMM40Ltranslocase ­of ­outer ­mitochondrial ­membrane ­40 ­likeTOMM40B, Q969M1, gene:8413425targetP signal, yeast mito homolog+, mito protein domain+, induction, coexpression+, MS/MSMOMProtein import, sorting and homeostasis > Protein import and sorting > TOMcerebrum, cerebellum, brainstem, spinalcord, adipose, largeintestine, stomach, testis
NDUFAF1NADH:ubiquinone ­oxidoreductase ­complex ­assembly ­factor ­1CGI-65, CGI65, CIA30, MC1DN11, Q9Y375, gene:5110325literature, APEX_matrix, targetP signal, mito protein domain, induction, coexpression++, MS/MS++MIMOXPHOS > Complex I > CI assembly factors;
OXPHOS > OXPHOS assembly factors
cerebellum, brainstem, spinalcord, heart, adipose, smallintestine, largeintestine, stomach, placenta, testis
NDUFB10NADH:ubiquinone ­oxidoreductase ­subunit ­B10PDSW, O96000, gene:471625literature, APEX_matrix, mito protein domain+, induction, coexpression++, MS/MS++MIMOXPHOS > Complex I > CI subunits;
OXPHOS > OXPHOS subunits
all 14
ACADSBacyl-CoA ­dehydrogenase ­short/branched ­chain2-MEBCAD, ACAD7, SBCAD, P45954, gene:3625literature, APEX_matrix, targetP signal, mito protein domain+, induction, coexpression, MS/MS++MatrixMetabolism > Lipid metabolism > Fatty acid oxidation;
Metabolism > Amino acid metabolism > BCAA metabolism
all 14
MRPS9mitochondrial ­ribosomal ­protein ­S9MRP-S9, RPMS9, S9mt, P82933, gene:6496525literature, APEX_matrix, targetP signal, yeast mito homolog++, Rickettsial homolog, mito protein domain, induction, coexpression, MS/MSMatrixMitochondrial central dogma > mtRNA metabolism > mtRNA granules;
Mitochondrial central dogma > Translation > Mitochondrial ribosome
all 14
COQ8Acoenzyme ­Q8AADCK3, ARCA2, CABC1, COQ10D4, COQ8, SCAR9, Q8NI60, gene:5699725literature, APEX_matrix, yeast mito homolog+, Rickettsial homolog, mito protein domain, induction, coexpression+, MS/MS++MIMMetabolism > Metals and cofactors > Coenzyme Q metabolismall 14
ACADVLacyl-CoA ­dehydrogenase ­very ­long ­chainACAD6, LCACD, VLCAD, P49748, gene:3725literature, APEX_matrix, targetP signal+, mito protein domain+, coexpression+, MS/MS++MIMMetabolism > Lipid metabolism > Fatty acid oxidationall 14
COQ4coenzyme ­Q4CGI-92, COQ10D7, Q9Y3A0, gene:5111725literature, targetP signal+, yeast mito homolog++, mito protein domain+, coexpression, MS/MSMIMMetabolism > Metals and cofactors > Coenzyme Q metabolismcerebrum, cerebellum, brainstem, kidney, liver, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta
MRPL15mitochondrial ­ribosomal ­protein ­L15HSPC145, L15mt, MRP-L15, MRP-L7, RPML7, Q9P015, gene:2908825literature, APEX_matrix, yeast mito homolog++, Rickettsial homolog, mito protein domain, induction, coexpression, MS/MSMatrixMitochondrial central dogma > Translation > Mitochondrial ribosomecerebrum, cerebellum, brainstem, spinalcord, kidney, liver, adipose, smallintestine, largeintestine, stomach, placenta, testis
NDUFA2NADH:ubiquinone ­oxidoreductase ­subunit ­A2B8, CD14, CIB8, MC1DN13, O43678, gene:469525literature, APEX_matrix, targetP signal, mito protein domain, induction, coexpression++, MS/MS++MIMOXPHOS > Complex I > CI subunits;
OXPHOS > OXPHOS subunits
all 14
MRPL24mitochondrial ­ribosomal ­protein ­L24L24mt, MRP-L18, MRP-L24, Q96A35, gene:7959025literature, APEX_matrix, targetP signal, yeast mito homolog++, Rickettsial homolog, mito protein domain, coexpression, MS/MS++MatrixMitochondrial central dogma > Translation > Mitochondrial ribosomecerebrum, cerebellum, brainstem, spinalcord, liver, heart, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
HSPD1heat ­shock ­protein ­family ­D ­(Hsp60) ­member ­1CPN60, GROEL, HLD4, HSP-60, HSP60, HSP65, HuCHA60, SPG13, P10809, gene:332925literature, APEX_matrix, targetP signal+, yeast mito homolog++, Rickettsial homolog, mito protein domain, MS/MS++MatrixProtein import, sorting and homeostasis > Protein homeostasis > Chaperonesall 14
NDUFS5NADH:ubiquinone ­oxidoreductase ­subunit ­S5CI-15k, CI15K, O43920, gene:472525literature, APEX_IMS, mito protein domain+, induction, coexpression++, MS/MS++MIMOXPHOS > Complex I > CI subunits;
OXPHOS > OXPHOS subunits
all 14
AK3adenylate ­kinase ­3AK3L1, AK6, AKL3L, AKL3L1, FIX, Q9UIJ7, gene:5080825literature, APEX_matrix, targetP signal, Rickettsial homolog, mito protein domain, induction, coexpression, MS/MS++MatrixMetabolism > Nucleotide metabolism > Nucleotide synthesis and processingall 14
CYCScytochrome ­c, ­somaticCYC, HCS, THC4, P99999, gene:5420524literature, APEX_IMS, yeast mito homolog++, Rickettsial homolog, mito protein domain+, MS/MS++IMSMetabolism > Metals and cofactors > Heme-containing proteins;
Metabolism > Electron carriers > Cytochromes;
OXPHOS > OXPHOS subunits;
OXPHOS > Cytochrome C;
Mitochondrial dynamics and surveillance > Apoptosis
all 14
MIPEPmitochondrial ­intermediate ­peptidaseCOXPD31, HMIP, MIP, Q99797, gene:428524literature, APEX_matrix, targetP signal+, yeast mito homolog++, mito protein domain, induction, MS/MS++MatrixProtein import, sorting and homeostasis > Protein import and sorting > Preprotein cleavage;
Protein import, sorting and homeostasis > Protein homeostasis > Proteases
cerebrum, spinalcord, kidney, liver, heart, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
LYRM7LYR ­motif ­containing ­7C5orf31, MC3DN8, MZM1L, Q5U5X0, gene:9062424literature, APEX_matrix, targetP signal, yeast mito homolog++, mito protein domain+, MS/MS++MatrixOXPHOS > Complex III > CIII assembly factors;
OXPHOS > OXPHOS assembly factors
cerebrum, cerebellum, brainstem, spinalcord, liver, heart, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
CRATcarnitine ­O-acetyltransferaseCAT, CAT1, NBIA8, P43155, gene:138424literature, APEX_matrix, targetP signal, yeast mito homolog+, mito protein domain, induction, coexpression, MS/MS++MIMMetabolism > Lipid metabolism > Fatty acid oxidationall 14
PCCBpropionyl-CoA ­carboxylase ­subunit ­betaP05166, gene:509624literature, targetP signal+, Rickettsial homolog, mito protein domain, coexpression+, MS/MS++MatrixMetabolism > Carbohydrate metabolism > Propanoate metabolism;
Metabolism > Lipid metabolism > Fatty acid oxidation
all 14
MRPS7mitochondrial ­ribosomal ­protein ­S7COXPD34, MRP-S, MRP-S7, RP-S7, RPMS7, S7mt, bMRP27a, Q9Y2R9, gene:5108124literature, APEX_matrix, targetP signal, yeast mito homolog++, Rickettsial homolog, mito protein domain, coexpression+, MS/MSMatrixMitochondrial central dogma > mtRNA metabolism > mtRNA granules;
Mitochondrial central dogma > Translation > Mitochondrial ribosome
all 14
MRPL3mitochondrial ­ribosomal ­protein ­L3COXPD9, MRL3, RPML3, P09001, gene:1122224literature, APEX_matrix, yeast mito homolog++, Rickettsial homolog, mito protein domain, induction, coexpressionMatrixMitochondrial central dogma > Translation > Mitochondrial ribosomecerebrum, cerebellum, brainstem, spinalcord, kidney, liver, heart, skeletalmuscle, adipose, smallintestine, largeintestine, placenta, testis
PRODHproline ­dehydrogenase ­1HSPOX2, PIG6, POX, PRODH1, PRODH2, TP53I6, O43272, gene:562524literature, targetP signal, yeast mito homolog+, mito protein domain+, coexpression, MS/MS++MIMMetabolism > Amino acid metabolism > Proline metabolism;
Metabolism > Electron carriers > Q-linked reactions, other
cerebrum, cerebellum, brainstem, spinalcord, kidney, liver, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
PCCApropionyl-CoA ­carboxylase ­subunit ­alphaP05165, gene:509524literature, targetP signal, Rickettsial homolog, mito protein domain, induction, coexpression+, MS/MS++MatrixMetabolism > Carbohydrate metabolism > Propanoate metabolism;
Metabolism > Lipid metabolism > Fatty acid oxidation;
Metabolism > Vitamin metabolism > Biotin utilizing proteins
all 14
MCCC1methylcrotonoyl-CoA ­carboxylase ­1MCC-B, MCCA, Q96RQ3, gene:5692224literature, targetP signal, Rickettsial homolog, mito protein domain, induction, coexpression+, MS/MS++MatrixMetabolism > Amino acid metabolism > BCAA metabolism;
Metabolism > Vitamin metabolism > Biotin utilizing proteins
all 14
MRPS12mitochondrial ­ribosomal ­protein ­S12MPR-S12, MT-RPS12, RPMS12, RPS12, RPSM12, O15235, gene:618324literature, yeast mito homolog++, Rickettsial homolog, mito protein domain, induction, coexpression+, MS/MS+MatrixMitochondrial central dogma > Translation > Mitochondrial ribosomecerebrum, cerebellum, brainstem, spinalcord, liver, heart, adipose, smallintestine, largeintestine, stomach, placenta, testis
CLPBcaseinolytic ­mitochondrial ­matrix ­peptidase ­chaperone ­subunit ­BANKCLB, HSP78, MEGCANN, MGCA7, SKD3, Q9H078, gene:8157024APEX_IMS, targetP signal+, yeast mito homolog+, Rickettsial homolog, mito protein domain, MS/MS++IMSProtein import, sorting and homeostasis > Protein homeostasis > Proteasescerebrum, cerebellum, brainstem, spinalcord, kidney, liver, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
PDK1pyruvate ­dehydrogenase ­kinase ­1Q15118, gene:516324literature, APEX_matrix, targetP signal+, yeast mito homolog+, mito protein domain+, MS/MS++MatrixMetabolism > Carbohydrate metabolism > Pyruvate metabolismall 14
MRPL49mitochondrial ­ribosomal ­protein ­L49C11orf4, L49mt, MRP-L49, NOF, NOF1, Q13405, gene:74024literature, APEX_matrix, targetP signal, yeast mito homolog++, mito protein domain, coexpression, MS/MS++MatrixMitochondrial central dogma > Translation > Mitochondrial ribosomeall 14
COX7A2cytochrome ­c ­oxidase ­subunit ­7A2COX7AL, COX7AL1, COXVIIAL, COXVIIa-L, VIIAL, P14406, gene:134724literature, mito protein domain+, induction, coexpression++, MS/MS++MIMOXPHOS > Complex IV > CIV subunits;
OXPHOS > OXPHOS subunits
all 14
TMEM126Atransmembrane ­protein ­126AOPA7, Q9H061, gene:8423324literature, mito protein domain+, induction, coexpression++, MS/MS++MIMOXPHOS > Complex I > CI assembly factors;
OXPHOS > OXPHOS assembly factors
all 14
ECHDC2enoyl-CoA ­hydratase ­domain ­containing ­2Q86YB7, gene:5526824targetP signal, yeast mito homolog+, Rickettsial homolog, mito protein domain, coexpression, MS/MS++Matrixnoneall 14
HCCSholocytochrome ­c ­synthaseCCHL, LSDMCA1, MCOPS7, MLS, P53701, gene:305224literature, APEX_IMS, yeast mito homolog++, mito protein domain+, induction, coexpression, MS/MSIMSMetabolism > Metals and cofactors > Heme synthesis and processing;
OXPHOS > OXPHOS subunits;
OXPHOS > Cytochrome C
cerebrum, spinalcord, heart, adipose, smallintestine, largeintestine, placenta, testis
HIBADH3-hydroxyisobutyrate ­dehydrogenaseNS5ATP1, P31937, gene:1111224literature, APEX_matrix, targetP signal+, mito protein domain, induction, coexpression+, MS/MS++MatrixMetabolism > Amino acid metabolism > BCAA metabolismall 14
MRPL19mitochondrial ­ribosomal ­protein ­L19L19mt, MRP-L15, MRP-L19, MRPL15, RLX1, RPML15, P49406, gene:980124literature, APEX_matrix, targetP signal, yeast mito homolog++, Rickettsial homolog, mito protein domain, coexpression, MS/MS+MatrixMitochondrial central dogma > Translation > Mitochondrial ribosomecerebrum, cerebellum, brainstem, spinalcord, liver, adipose, smallintestine, largeintestine, stomach, placenta, testis
MRPL36mitochondrial ­ribosomal ­protein ­L36BRIP1, L36mt, MRP-L36, PRPL36, RPMJ, Q9P0J6, gene:6497924literature, targetP signal, yeast mito homolog++, Rickettsial homolog, mito protein domain, induction, coexpression+MatrixMitochondrial central dogma > Translation > Mitochondrial ribosomenone
SLC25A30solute ­carrier ­family ­25 ­member ­30KMCP1, Q5SVS4, gene:25351224literature, yeast mito homolog+, mito protein domain+, coexpression, MS/MS++MIMMetabolism > Sulfur metabolism;
Small molecule transport > SLC25A family
cerebrum, cerebellum, liver, placenta, testis
BDH13-hydroxybutyrate ­dehydrogenase ­1BDH, SDR9C1, Q02338, gene:62224literature, APEX_matrix, targetP signal+, Rickettsial homolog, mito protein domain, induction, coexpression, MS/MS++MIMMetabolism > Carbohydrate metabolism > Ketone metabolismall 14
FARS2phenylalanyl-tRNA ­synthetase ­2, ­mitochondrialCOXPD14, FARS1, HSPC320, PheRS, SPG77, mtPheRS, O95363, gene:1066723literature, APEX_matrix, targetP signal, yeast mito homolog+, Rickettsial homolog, mito protein domain, induction, coexpression, MS/MSMatrixMitochondrial central dogma > Translation > mt-tRNA synthetasesadipose, largeintestine, stomach, placenta, testis
ABCB7ATP ­binding ­cassette ­subfamily ­B ­member ­7ABC7, ASAT, Atm1p, EST140535, O75027, gene:2223literature, APEX_matrix, targetP signal, yeast mito homolog+, Rickettsial homolog, mito protein domain, coexpression+, MS/MS+MIMMetabolism > Metals and cofactors > Heme synthesis and processing;
Metabolism > Metals and cofactors > Fe-S cluster biosynthesis;
Metabolism > Metals and cofactors > Molybdenum cofactor synthesis and proteins;
Small molecule transport > ABC transporters
all 14
MTX1metaxin ­1MTX, MTXN, Q13505, gene:458023literature, yeast mito homolog++, mito protein domain+, coexpression, MS/MS++MOMProtein import, sorting and homeostasis > Protein import and sorting > SAM;
Mitochondrial dynamics and surveillance > Intramitochondrial membrane interactions
all 14
NDUFA7NADH:ubiquinone ­oxidoreductase ­subunit ­A7B14.5a, CI-B14.5a, O95182, gene:470123literature, APEX_matrix, mito protein domain+, coexpression++, MS/MS++MIMOXPHOS > Complex I > CI subunits;
OXPHOS > OXPHOS subunits
all 14
TIMM17Atranslocase ­of ­inner ­mitochondrial ­membrane ­17ATIM17, TIM17A, Q99595, gene:1044023literature, APEX_IMS, yeast mito homolog+, mito protein domain+, induction, coexpression, MS/MS+MIMProtein import, sorting and homeostasis > Protein import and sorting > TIM23 presequence pathwayall 14
ALDH9A1aldehyde ­dehydrogenase ­9 ­family ­member ­A1ALDH4, ALDH7, ALDH9, E3, TMABA-DH, TMABADH, TMABALDH, P49189, gene:22323APEX_matrix, targetP signal+, yeast mito homolog+, mito protein domain, coexpression, MS/MS++MatrixMetabolism > Amino acid metabolism > GABA metabolism;
Metabolism > Detoxification > ROS and glutathione metabolism
brainstem, spinalcord, kidney, liver, adipose, smallintestine, largeintestine, stomach, placenta, testis
MRPS18Cmitochondrial ­ribosomal ­protein ­S18CCGI-134, MRP-S18-1, MRP-S18-c, MRPS18-1, S18mt-c, mrps18-c, Q9Y3D5, gene:5102323literature, yeast mito homolog++, Rickettsial homolog, mito protein domain, coexpression, MS/MS++MatrixMitochondrial central dogma > Translation > Mitochondrial ribosomecerebrum, cerebellum, brainstem, spinalcord, liver, heart, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
MARS2methionyl-tRNA ­synthetase ­2, ­mitochondrialCOXPD25, MetRS, mtMetRS, Q96GW9, gene:9293523literature, APEX_matrix, targetP signal, yeast mito homolog++, Rickettsial homolog, mito protein domain, coexpression, MS/MS+MatrixMitochondrial central dogma > Translation > mt-tRNA synthetasescerebrum, cerebellum, brainstem, spinalcord, heart, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
ALDH6A1aldehyde ­dehydrogenase ­6 ­family ­member ­A1MMSADHA, MMSDH, Q02252, gene:432923literature, APEX_matrix, targetP signal, yeast mito homolog+, mito protein domain, coexpression, MS/MS++MIMMetabolism > Amino acid metabolism > BCAA metabolismall 14
FDXRferredoxin ­reductaseADR, ADXR, ANOA, P22570, gene:223223literature, APEX_matrix, targetP signal+, yeast mito homolog++, coexpression, MS/MS++MatrixMetabolism > Lipid metabolism > Cholesterol, bile acid, steroid synthesis;
Metabolism > Metals and cofactors > Heme synthesis and processing;
Metabolism > Metals and cofactors > Fe-S cluster biosynthesis;
Metabolism > Vitamin metabolism > Vitamin D metabolism
spinalcord, kidney, liver, adipose, smallintestine, largeintestine, stomach, placenta, testis
GATCglutamyl-tRNA ­amidotransferase ­subunit ­C15E1.2, COXPD42, O43716, gene:28345923literature, APEX_matrix, targetP signal, Rickettsial homolog, mito protein domain+, induction, coexpression, MS/MSMatrixMitochondrial central dogma > Translation > mt-tRNA synthetasescerebrum, cerebellum, spinalcord, kidney, largeintestine, testis
TRAP1TNF ­receptor ­associated ­protein ­1HSP 75, HSP75, HSP90L, TRAP-1, Q12931, gene:1013123APEX_matrix, GFP, targetP signal+, Rickettsial homolog, mito protein domain, coexpression++, MS/MS+MatrixProtein import, sorting and homeostasis > Protein homeostasis > Chaperonesall 14
ACAD8acyl-CoA ­dehydrogenase ­family ­member ­8ACAD-8, ARC42, IBDH, Q9UKU7, gene:2703422literature, APEX_matrix, targetP signal+, mito protein domain+, coexpression, MS/MS++MatrixMetabolism > Amino acid metabolism > BCAA metabolismall 14
ALDH2aldehyde ­dehydrogenase ­2 ­family ­memberALDH-E2, ALDHI, ALDM, P05091, gene:21722literature, APEX_matrix, targetP signal+, yeast mito homolog+, mito protein domain, coexpression, MS/MS++MatrixMetabolism > Detoxification > Xenobiotic metabolismall 14
PPIFpeptidylprolyl ­isomerase ­FCYP3, CyP-M, Cyp-D, CypD, P30405, gene:1010522literature, APEX_matrix, targetP signal+, mito protein domain, induction, coexpression, MS/MS++MatrixSignaling > Calcium homeostasis > Mitochondrial permeability transition poreall 14
TIMM22translocase ­of ­inner ­mitochondrial ­membrane ­22COXPD43, TEX4, TIM22, Q9Y584, gene:2992822literature, yeast mito homolog++, mito protein domain+, induction, coexpression, MS/MSMIMProtein import, sorting and homeostasis > Protein import and sorting > TIM22 carrier pathwayall 14
IVDisovaleryl-CoA ­dehydrogenaseACAD2, P26440, gene:371222literature, APEX_matrix, targetP signal, mito protein domain+, coexpression+, MS/MS++MatrixMetabolism > Amino acid metabolism > BCAA metabolismall 14
L2HGDHL-2-hydroxyglutarate ­dehydrogenaseC14orf160, L2HGA, Q9H9P8, gene:7994422literature, APEX_matrix, targetP signal+, mito protein domain, induction, coexpression, MS/MS++MIMMetabolism > Carbohydrate metabolism > TCA-associatedcerebrum, spinalcord, kidney, liver, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, testis
ETHE1ETHE1 ­persulfide ­dioxygenaseHSCO, YF13H12, O95571, gene:2347422literature, APEX_matrix, targetP signal, yeast mito homolog+, mito protein domain, induction, MS/MS++MatrixMetabolism > Sulfur metabolismcerebrum, cerebellum, brainstem, spinalcord, kidney, liver, heart, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta
MRPL20mitochondrial ­ribosomal ­protein ­L20L20mt, MRP-L20, Q9BYC9, gene:5505222literature, APEX_matrix, targetP signal+, Rickettsial homolog, mito protein domain, induction, coexpression, MS/MSMatrixMitochondrial central dogma > Translation > Mitochondrial ribosomecerebrum, cerebellum, brainstem, spinalcord, liver, heart, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
SLC25A5solute ­carrier ­family ­25 ­member ­52F1, AAC2, ANT2, T2, T3, P05141, gene:29222literature, yeast mito homolog+, mito protein domain+, induction, MS/MS++MIMMetabolism > Nucleotide metabolism > Nucleotide import;
Signaling > Calcium homeostasis > Mitochondrial permeability transition pore;
Small molecule transport > SLC25A family
all 14
SLC25A12solute ­carrier ­family ­25 ­member ­12AGC1, ARALAR, EIEE39, O75746, gene:860422literature, yeast mito homolog+, mito protein domain+, induction, MS/MS++MIMMetabolism > Carbohydrate metabolism > Malate-aspartate shuttle;
Metabolism > Amino acid metabolism > Glutamate metabolism;
Signaling > Calcium homeostasis > EF hand proteins;
Small molecule transport > SLC25A family
all 14
MRPS21mitochondrial ­ribosomal ­protein ­S21MDS016, MRP-S21, RPMS21, P82921, gene:5446022literature, yeast mito homolog++, Rickettsial homolog, mito protein domain, induction, MS/MS++MatrixMitochondrial central dogma > Translation > Mitochondrial ribosomeall 14
TOMM22translocase ­of ­outer ­mitochondrial ­membrane ­221C9-2, MST065, MSTP065, TOM22, Q9NS69, gene:5699322literature, yeast mito homolog++, mito protein domain+, coexpression, MS/MS++MOMProtein import, sorting and homeostasis > Protein import and sorting > TOMall 14
ACAA2acetyl-CoA ­acyltransferase ­2DSAEC, P42765, gene:1044922literature, APEX_matrix, targetP signal, Rickettsial homolog, mito protein domain, induction, coexpression+, MS/MS++MatrixMetabolism > Carbohydrate metabolism > Ketone metabolism;
Metabolism > Lipid metabolism > Fatty acid oxidation;
Metabolism > Amino acid metabolism > Lysine metabolism
all 14
MRPL30mitochondrial ­ribosomal ­protein ­L30L28MT, L30MT, MRP-L28, MRP-L30, MRPL28, MRPL28M, RPML28, Q8TCC3, gene:5126322literature, APEX_matrix, yeast mito homolog++, mito protein domain, coexpression++, MS/MSMatrixMitochondrial central dogma > Translation > Mitochondrial ribosomeall 14
DNAJA3DnaJ ­heat ­shock ­protein ­family ­(Hsp40) ­member ­A3HCA57, TID1, hTID-1, Q96EY1, gene:909322literature, APEX_matrix, targetP signal, Rickettsial homolog, mito protein domain, induction, coexpression++, MS/MS+MatrixProtein import, sorting and homeostasis > Protein homeostasis > Chaperonesall 14
NDUFB2NADH:ubiquinone ­oxidoreductase ­subunit ­B2AGGG, CI-AGGG, O95178, gene:470822literature, targetP signal, induction, coexpression++, MS/MS++MIMOXPHOS > Complex I > CI subunits;
OXPHOS > OXPHOS subunits
all 14
MRPS34mitochondrial ­ribosomal ­protein ­S34COXPD32, MRP-S12, MRP-S34, MRPS12, P82930, gene:6599322literature, APEX_matrix, targetP signal, induction, coexpression++, MS/MS++MatrixMitochondrial central dogma > Translation > Mitochondrial ribosomeall 14
ETFBelectron ­transfer ­flavoprotein ­subunit ­betaFP585, MADD, P38117, gene:210922literature, APEX_matrix, yeast mito homolog, mito protein domain, induction, coexpression+, MS/MS++MatrixMetabolism > Lipid metabolism > Fatty acid oxidation;
Metabolism > Amino acid metabolism > BCAA metabolism;
Metabolism > Amino acid metabolism > Lysine metabolism;
Metabolism > Amino acid metabolism > Glycine metabolism;
Metabolism > Vitamin metabolism > Choline and betaine metabolism;
Metabolism > Electron carriers > Q-linked reactions, other
all 14
AFG1LAFG1 ­like ­ATPaseAFG1, LACE1, c222389, Q8WV93, gene:24626922APEX_matrix, yeast mito homolog++, Rickettsial homolog, coexpression, MS/MS+MIMProtein import, sorting and homeostasis > Protein homeostasis > Proteasescerebrum, cerebellum, spinalcord, heart, adipose, smallintestine, largeintestine, stomach, placenta
ATP5IF1ATP ­synthase ­inhibitory ­factor ­subunit ­1ATPI, ATPIF1, ATPIP, IP, Q9UII2, gene:9397422literature, APEX_matrix, targetP signal, yeast mito homolog++, mito protein domain+, MS/MS++MatrixOXPHOS > Complex V > CV subunits;
OXPHOS > OXPHOS subunits
all 14
ATP5PBATP ­synthase ­peripheral ­stalk-membrane ­subunit ­bATP5F1, PIG47, P24539, gene:51522literature, APEX_matrix, yeast mito homolog++, mito protein domain+, MS/MS++MIMOXPHOS > Complex V > CV subunits;
OXPHOS > OXPHOS subunits
all 14
COX7Ccytochrome ­c ­oxidase ­subunit ­7CP15954, gene:135022literature, APEX_IMS, yeast mito homolog++, mito protein domain+, MS/MS++MIMOXPHOS > Complex IV > CIV subunits;
OXPHOS > OXPHOS subunits
all 14
CHCHD3coiled-coil-helix-coiled-coil-helix ­domain ­containing ­3MICOS19, MINOS3, Mic19, PPP1R22, Q9NX63, gene:5492722literature, APEX_IMS, APEX_matrix, GFP, yeast mito homolog, induction, coexpression++, MS/MS++MIMMitochondrial dynamics and surveillance > Cristae formation > MICOS complex;
Mitochondrial dynamics and surveillance > Intramitochondrial membrane interactions
all 14
COQ10Bcoenzyme ­Q10BQ9H8M1, gene:8021922GFP, targetP signal, yeast mito homolog+, Rickettsial homolog, mito protein domain+, MS/MS+MIMMetabolism > Metals and cofactors > Coenzyme Q metabolismcerebrum, cerebellum, brainstem, spinalcord, liver, heart, skeletalmuscle, adipose, largeintestine, stomach, placenta
ACSF3acyl-CoA ­synthetase ­family ­member ­3Q4G176, gene:19732222APEX_matrix, targetP signal+, Rickettsial homolog, mito protein domain, coexpression+, MS/MS++MatrixMetabolism > Lipid metabolism > Type II fatty acid synthesisall 14
POLDIP2DNA ­polymerase ­delta ­interacting ­protein ­2PDIP38, POLD4, p38, Q9Y2S7, gene:2607322literature, APEX_matrix, targetP signal+, induction, coexpression++, MS/MS++MatrixMitochondrial central dogma > mtDNA maintenance > mtDNA nucleoidall 14
SLC25A10solute ­carrier ­family ­25 ­member ­10DIC, MTDPS19, Q9UBX3, gene:146822literature, yeast mito homolog+, mito protein domain+, coexpression, MS/MS++MIMMetabolism > Carbohydrate metabolism > Gluconeogenesis;
Metabolism > Carbohydrate metabolism > Malate-aspartate shuttle;
Small molecule transport > SLC25A family
cerebrum, cerebellum, brainstem, spinalcord, kidney, liver, adipose, smallintestine, largeintestine, stomach, placenta, testis
SLC25A13solute ­carrier ­family ­25 ­member ­13ARALAR2, CITRIN, CTLN2, NICCD, Q9UJS0, gene:1016522literature, yeast mito homolog+, mito protein domain+, coexpression, MS/MS++MIMMetabolism > Carbohydrate metabolism > Malate-aspartate shuttle;
Metabolism > Amino acid metabolism > Urea cycle;
Signaling > Calcium homeostasis > EF hand proteins;
Small molecule transport > SLC25A family
all 14
PDK3pyruvate ­dehydrogenase ­kinase ­3CMTX6, GS1-358P8.4, Q15120, gene:516521literature, APEX_matrix, targetP signal, yeast mito homolog+, mito protein domain+, MS/MS++MatrixMetabolism > Carbohydrate metabolism > Pyruvate metabolismcerebrum, cerebellum, brainstem, spinalcord, kidney, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
ME3malic ­enzyme ­3NADP-ME, Q16798, gene:1087321literature, yeast mito homolog+, Rickettsial homolog, mito protein domain, coexpression, MS/MS++MatrixMetabolism > Carbohydrate metabolism > TCA-associatedcerebrum, cerebellum, brainstem, spinalcord, kidney, heart, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
MRPL22mitochondrial ­ribosomal ­protein ­L22HSPC158, L22mt, MRP-L22, MRP-L25, RPML25, Q9NWU5, gene:2909321literature, APEX_matrix, targetP signal, yeast mito homolog++, Rickettsial homolog, mito protein domain, MS/MS++MatrixMitochondrial central dogma > Translation > Mitochondrial ribosomeall 14
IDH2isocitrate ­dehydrogenase ­(NADP(+)) ­2D2HGA2, ICD-M, IDH, IDHM, IDP, IDPM, mNADP-IDH, P48735, gene:341821literature, APEX_matrix, targetP signal+, Rickettsial homolog, mito protein domain, induction, coexpression, MS/MS++MatrixMetabolism > Carbohydrate metabolism > TCA cycleall 14
GCDHglutaryl-CoA ­dehydrogenaseACAD5, GCD, Q92947, gene:263921literature, APEX_matrix, targetP signal+, mito protein domain+, coexpression, MS/MS++MatrixMetabolism > Amino acid metabolism > Lysine metabolismall 14
MRPL47mitochondrial ­ribosomal ­protein ­L47CGI-204, L47mt, MRP-L47, NCM1, Q9HD33, gene:5712921literature, APEX_matrix, targetP signal+, yeast mito homolog++, mito protein domain, induction, coexpressionMatrixMitochondrial central dogma > mtRNA metabolism > mtRNA granules;
Mitochondrial central dogma > Translation > Mitochondrial ribosome
all 14
PPA2inorganic ­pyrophosphatase ­2HSPC124, SCFAI, SCFI, SID6-306, Q9H2U2, gene:2706821literature, APEX_matrix, targetP signal+, Rickettsial homolog, mito protein domain, coexpression, MS/MS++MatrixMitochondrial central dogma > mtDNA maintenance;
Mitochondrial central dogma > mtRNA metabolism;
Mitochondrial central dogma > Translation;
Metabolism > Nucleotide metabolism
all 14
MRPL9mitochondrial ­ribosomal ­protein ­L9L9mt, Q9BYD2, gene:6500521literature, APEX_matrix, targetP signal, Rickettsial homolog, mito protein domain, induction, coexpression+, MS/MSMatrixMitochondrial central dogma > Translation > Mitochondrial ribosomeall 14
CHCHD10coiled-coil-helix-coiled-coil-helix ­domain ­containing ­10C22orf16, FTDALS2, IMMD, MIX17A, N27C7-4, SMAJ, Q8WYQ3, gene:40091621literature, targetP signal, yeast mito homolog+, mito protein domain, induction, coexpression++IMSnonenone
WARS2tryptophanyl ­tRNA ­synthetase ­2, ­mitochondrialNEMMLAS, TrpRS, mtTrpRS, Q9UGM6, gene:1035221literature, APEX_matrix, targetP signal, yeast mito homolog++, Rickettsial homolog, mito protein domain, coexpression, MS/MSMatrixMitochondrial central dogma > Translation > mt-tRNA synthetasescerebrum, cerebellum, brainstem, kidney, skeletalmuscle, adipose, largeintestine, placenta, testis
SLC25A19solute ­carrier ­family ­25 ­member ­19DNC, MCPHA, MUP1, THMD3, THMD4, TPC, Q9HC21, gene:6038621literature, yeast mito homolog++, mito protein domain+, induction, coexpression, MS/MSMIMMetabolism > Vitamin metabolism > Vitamin B1 metabolism;
Small molecule transport > SLC25A family
cerebellum, spinalcord, adipose, testis
CBR4carbonyl ­reductase ­4SDR45C1, Q8N4T8, gene:8486921literature, APEX_matrix, Rickettsial homolog, mito protein domain, induction, coexpression+, MS/MS++MatrixMetabolism > Lipid metabolism > Type II fatty acid synthesisall 14
SMDT1single-pass ­membrane ­protein ­with ­aspartate ­rich ­tail ­1C22orf32, DDDD, EMRE, Q9H4I9, gene:9168921literature, targetP signal, mito protein domain+, induction, coexpression+, MS/MSMIMSignaling > Calcium homeostasis > Calcium cycle;
Small molecule transport > Calcium uniporter
cerebrum, cerebellum, brainstem, spinalcord, kidney, adipose, smallintestine, largeintestine, stomach, placenta, testis
HAGHhydroxyacylglutathione ­hydrolaseGLO2, GLX2, GLXII, HAGH1, Q16775, gene:302921literature, APEX_matrix, targetP signal+, mito protein domain, induction, coexpression, MS/MS++MatrixMetabolism > Detoxification > ROS and glutathione metabolismcerebrum, cerebellum, brainstem, spinalcord, kidney, liver, adipose, smallintestine, largeintestine, stomach, placenta, testis
COX7A1cytochrome ­c ­oxidase ­subunit ­7A1COX7A, COX7AH, COX7AM, P24310, gene:134621literature, targetP signal, mito protein domain+, induction, coexpression, MS/MS+MIMOXPHOS > Complex IV > CIV subunits;
OXPHOS > OXPHOS subunits
cerebrum, brainstem, spinalcord, kidney, liver, heart, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
MTG1mitochondrial ­ribosome ­associated ­GTPase ­1GTP, GTPBP7, Q9BT17, gene:9217021literature, APEX_matrix, targetP signal, yeast mito homolog++, Rickettsial homolog, mito protein domain, coexpression+, MS/MSMatrixMitochondrial central dogma > Translation > Mitochondrial ribosome assemblylargeintestine, testis
COX14cytochrome ­c ­oxidase ­assembly ­factor ­COX14C12orf62, PCAG1, Q96I36, gene:8498721literature, GFP, mito protein domain+, induction, coexpression++, MS/MS+MIMMitochondrial central dogma > Translation > Translation factors;
OXPHOS > Complex IV > CIV assembly factors;
OXPHOS > OXPHOS assembly factors
cerebrum, cerebellum, brainstem, spinalcord, kidney, heart, smallintestine, testis
NDUFA12NADH:ubiquinone ­oxidoreductase ­subunit ­A12B17.2, DAP13, MC1DN23, Q9UI09, gene:5596721literature, APEX_matrix, Rickettsial homolog, coexpression++, MS/MS++MIMOXPHOS > Complex I > CI subunits;
OXPHOS > OXPHOS subunits
all 14
MRPS16mitochondrial ­ribosomal ­protein ­S16CGI-132, COXPD2, MRP-S16, RPMS16, Q9Y3D3, gene:5102121literature, APEX_matrix, targetP signal, yeast mito homolog++, Rickettsial homolog, mito protein domain, MS/MS+MatrixMitochondrial central dogma > Translation > Mitochondrial ribosomeall 14
MTERF2mitochondrial ­transcription ­termination ­factor ­2MTERFD3, mTERFL, Q49AM1, gene:8029821literature, APEX_matrix, targetP signal+, mito protein domain+, induction, coexpression, MS/MSMatrixMitochondrial central dogma > mtDNA maintenance > mtDNA nucleoidcerebrum, cerebellum, brainstem, spinalcord, liver, heart, skeletalmuscle, adipose, largeintestine, testis
ETFRF1electron ­transfer ­flavoprotein ­regulatory ­factor ­1LYRM5, Q6IPR1, gene:14436321mito protein domain+, induction, coexpression, MS/MS++MatrixMetabolism > Electron carriers > Q-linked reactions, othercerebrum, brainstem, spinalcord, kidney, liver, heart, adipose, smallintestine, largeintestine, stomach, placenta
GPD2glycerol-3-phosphate ­dehydrogenase ­2GDH2, GPDM, mGDH, mGPDH, P43304, gene:282021literature, APEX_IMS, yeast mito homolog++, mito protein domain, coexpression, MS/MS++MIMMetabolism > Carbohydrate metabolism > Glycerol phosphate shuttle;
Metabolism > Electron carriers > Q-linked reactions, other
cerebrum, cerebellum, brainstem, spinalcord, kidney, liver, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
NDUFA10NADH:ubiquinone ­oxidoreductase ­subunit ­A10CI-42KD, CI-42k, MC1DN22, O95299, gene:470521literature, targetP signal, mito protein domain, coexpression++, MS/MS++MIMOXPHOS > Complex I > CI subunits;
OXPHOS > OXPHOS subunits
all 14
NDUFC2NADH:ubiquinone ­oxidoreductase ­subunit ­C2B14.5b, CI-B14.5b, HLC-1, NADHDH2, O95298, gene:471821literature, APEX_IMS, mito protein domain+, coexpression++, MS/MS++MIMOXPHOS > Complex I > CI subunits;
OXPHOS > OXPHOS subunits
all 14
SLIRPSRA ­stem-loop ­interacting ­RNA ­binding ­proteinC14orf156, DC50, PD04872, Q9GZT3, gene:8189221literature, APEX_matrix, GFP, targetP signal+, mito protein domain, induction, coexpression, MS/MS++MatrixMitochondrial central dogma > mtRNA metabolism > mtRNA stability and decay;
Mitochondrial central dogma > Translation
all 14
MT-ATP6ATP ­synthase ­F0 ­subunit ­6ATPase6, MTATP6, P00846, gene:450821literature, yeast mito homolog++, Rickettsial homolog, mito protein domain+, MS/MS+MIMOXPHOS > Complex V > CV subunits;
OXPHOS > OXPHOS subunits
all 14
MT-CO2cytochrome ­c ­oxidase ­subunit ­IICOII, MTCO2, P00403, gene:451321literature, APEX_IMS, APEX_matrix, yeast mito homolog++, Rickettsial homolog, mito protein domain+, MS/MS+MIMOXPHOS > Complex IV > CIV subunits;
OXPHOS > OXPHOS subunits
all 14
TIMM21translocase ­of ­inner ­mitochondrial ­membrane ­21C18orf55, HSPC154, TIM21, Q9BVV7, gene:2909021APEX_IMS, GFP, yeast mito homolog++, mito protein domain+, coexpression, MS/MSMIMMitochondrial central dogma > Translation > Translation factors;
Protein import, sorting and homeostasis > Protein import and sorting > TIM23 presequence pathway;
OXPHOS > Complex IV > CIV assembly factors;
OXPHOS > OXPHOS assembly factors
cerebrum, cerebellum, brainstem, spinalcord, liver, heart, skeletalmuscle, adipose, largeintestine, stomach, placenta, testis
NDUFA8NADH:ubiquinone ­oxidoreductase ­subunit ­A8CI-19KD, CI-PGIV, PGIV, P51970, gene:470221literature, APEX_IMS, mito protein domain, induction, coexpression++, MS/MS++MIMOXPHOS > Complex I > CI subunits;
OXPHOS > OXPHOS subunits
all 14
GLSglutaminaseAAD20, CASGID, EIEE71, GAC, GAM, GDPAG, GLS1, KGA, O94925, gene:274421literature, APEX_matrix, targetP signal+, Rickettsial homolog, mito protein domain+, MS/MS++MIMMetabolism > Amino acid metabolism > Glutamate metabolismcerebrum, cerebellum, brainstem, spinalcord, kidney, heart, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
NDUFB11NADH:ubiquinone ­oxidoreductase ­subunit ­B11CI-ESSS, ESSS, MC1DN30, NP17.3, Np15, P17.3, Q9NX14, gene:5453920literature, APEX_IMS, APEX_matrix, targetP signal+, coexpression++, MS/MS++MIMOXPHOS > Complex I > CI subunits;
OXPHOS > OXPHOS subunits
all 14
DHTKD1dehydrogenase ­E1 ­and ­transketolase ­domain ­containing ­1AMOXAD, CMT2Q, Q96HY7, gene:5552620APEX_matrix, targetP signal, yeast mito homolog+, Rickettsial homolog, mito protein domain+, MS/MSMatrixMetabolism > Amino acid metabolism > Lysine metabolismcerebrum, kidney, liver, adipose, placenta, testis
FAHD2Afumarylacetoacetate ­hydrolase ­domain ­containing ­2ACGI-105, Q96GK7, gene:5101120APEX_matrix, targetP signal, mito protein domain, induction, coexpression, MS/MS++Matrixnoneall 14
HSD17B8hydroxysteroid ­17-beta ­dehydrogenase ­8D6S2245E, FABG, FABGL, H2-KE6, HKE6, KE6, RING2, SDR30C1, dJ1033B10.9, Q92506, gene:792320APEX_matrix, targetP signal+, Rickettsial homolog, mito protein domain, coexpression, MS/MS++MatrixMetabolism > Lipid metabolism > Type II fatty acid synthesis;
Metabolism > Lipid metabolism > Cholesterol, bile acid, steroid synthesis
all 14
HINT2histidine ­triad ­nucleotide ­binding ­protein ­2HIT-17, Q9BX68, gene:8468120APEX_matrix, targetP signal+, Rickettsial homolog, mito protein domain, coexpression, MS/MS++MatrixMetabolism > Lipid metabolism > Cholesterol, bile acid, steroid synthesisall 14
MRPS5mitochondrial ­ribosomal ­protein ­S5MRP-S5, S5mt, P82675, gene:6496920literature, APEX_matrix, yeast mito homolog++, Rickettsial homolog, mito protein domain, induction, coexpression, MS/MSMatrixMitochondrial central dogma > Translation > Mitochondrial ribosomecerebrum, kidney, liver, heart, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
C6orf136chromosome ­6 ­open ­reading ­frame ­136Q5SQH8, gene:22154520targetP signal, induction, coexpression++, MS/MS+Matrixnoneall 14
SPRYD4SPRY ­domain ­containing ­4Q8WW59, gene:28337720APEX_matrix, GFP, targetP signal+, mito protein domain, coexpression+, MS/MS++Matrixnoneall 14
LIPT2lipoyl(octanoyl) ­transferase ­2A6NK58, gene:38778720targetP signal, yeast mito homolog, Rickettsial homolog, mito protein domain, coexpression, MS/MSMatrixMetabolism > Lipid metabolism > Lipoate insertioncerebrum, cerebellum, brainstem, spinalcord, skeletalmuscle, adipose, largeintestine, placenta, testis
DECR12,4-dienoyl-CoA ­reductase ­1DECR, NADPH, SDR18C1, Q16698, gene:166620literature, APEX_matrix, targetP signal, Rickettsial homolog, mito protein domain, coexpression+, MS/MS++MatrixMetabolism > Lipid metabolism > Fatty acid oxidationall 14
MRPS26mitochondrial ­ribosomal ­protein ­S26C20orf193, GI008, MRP-S13, MRP-S26, MRPS13, NY-BR-87, RPMS13, dJ534B8.3, Q9BYN8, gene:6494920literature, APEX_matrix, targetP signal, mito protein domain+, induction, coexpression, MS/MS+MatrixMitochondrial central dogma > Translation > Mitochondrial ribosomeall 14
SLC25A15solute ­carrier ­family ­25 ­member ­15D13S327, HHH, LNC-HC, ORC1, ORNT1, Q9Y619, gene:1016620literature, yeast mito homolog+, mito protein domain+, coexpression, MS/MS++MIMMetabolism > Amino acid metabolism > Urea cycle;
Small molecule transport > SLC25A family
spinalcord, kidney, liver, adipose, smallintestine, largeintestine, placenta
NDUFV3NADH:ubiquinone ­oxidoreductase ­subunit ­V3CI-10k, CI-9KD, P56181, gene:473120literature, APEX_matrix, targetP signal, induction, coexpression++, MS/MS++MIMOXPHOS > Complex I > CI subunits;
OXPHOS > OXPHOS subunits
all 14
BPHLbiphenyl ­hydrolase ­likeBPH-RP, MCNAA, VACVASE, Q86WA6, gene:67020targetP signal+, Rickettsial homolog, mito protein domain, coexpression, MS/MS++MatrixMetabolism > Detoxification > Xenobiotic metabolismall 14
STOML2stomatin ­like ­2HSPC108, SLP-2, Q9UJZ1, gene:3096820literature, APEX_matrix, targetP signal+, Rickettsial homolog, mito protein domain, coexpression, MS/MS++MIMProtein import, sorting and homeostasis > Protein homeostasisall 14
MSRB2methionine ­sulfoxide ­reductase ­B2CBS-1, CBS1, CGI-131, MSRB, PILB, Q9Y3D2, gene:2292120APEX_matrix, targetP signal, yeast mito homolog+, mito protein domain, coexpression, MS/MS++MatrixMetabolism > Detoxification > ROS and glutathione metabolism;
Metabolism > Sulfur metabolism
cerebrum, cerebellum, brainstem, spinalcord, kidney, liver, heart, skeletalmuscle, adipose, largeintestine, stomach, placenta, testis
LACTBlactamase ­betaG24, MRPL56, P83111, gene:11429420literature, APEX_IMS, targetP signal+, Rickettsial homolog, mito protein domain, coexpression, MS/MS++IMSProtein import, sorting and homeostasis > Protein homeostasis > Proteases;
Metabolism > Lipid metabolism > Phospholipid metabolism
all 14
TOMM40translocase ­of ­outer ­mitochondrial ­membrane ­40C19orf1, D19S1177E, PER-EC1, PEREC1, TOM40, O96008, gene:1045220literature, yeast mito homolog+, mito protein domain+, coexpression, MS/MS++MOMProtein import, sorting and homeostasis > Protein import and sorting > TOMcerebrum, spinalcord, kidney, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
SLC25A25solute ­carrier ­family ­25 ­member ­25MCSC, PCSCL, SCAMC-2, Q6KCM7, gene:11478920literature, yeast mito homolog+, mito protein domain+, coexpression, MS/MS++MIMMetabolism > Nucleotide metabolism > Nucleotide import;
Signaling > Calcium homeostasis > EF hand proteins;
Small molecule transport > SLC25A family
cerebrum, brainstem, spinalcord, liver, adipose, smallintestine, largeintestine, placenta
ADCK5aarF ­domain ­containing ­kinase ­5Q3MIX3, gene:20305420targetP signal, yeast mito homolog+, Rickettsial homolog, mito protein domain, induction, coexpression, MS/MSunknownnonecerebrum, largeintestine, testis
ACAD10acyl-CoA ­dehydrogenase ­family ­member ­10Q6JQN1, gene:8072420APEX_matrix, targetP signal+, mito protein domain+, coexpression, MS/MS++MatrixMetabolism > Lipid metabolism > Fatty acid oxidationcerebrum, spinalcord, kidney, liver, heart, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
VWA8von ­Willebrand ­factor ­A ­domain ­containing ­8KIAA0564, P7BP2, A3KMH1, gene:2307820APEX_matrix, targetP signal, mito protein domain, coexpression+, MS/MS++Matrixnoneall 14
CCDC90Bcoiled-coil ­domain ­containing ­90BMDS011, MDS025, Q9GZT6, gene:6049220literature, APEX_matrix, targetP signal+, yeast mito homolog+, mito protein domain+, MS/MSMIMnoneall 14
DNAJC11DnaJ ­heat ­shock ­protein ­family ­(Hsp40) ­member ­C11dJ126A5.1, Q9NVH1, gene:5573520GFP, yeast mito homolog+, Rickettsial homolog, mito protein domain, coexpression, MS/MS++MOMProtein import, sorting and homeostasis > Protein import and sorting;
Mitochondrial dynamics and surveillance > Intramitochondrial membrane interactions
all 14
ALKBH7alkB ­homolog ­7ABH7, SPATA11, UNQ6002, Q9BT30, gene:8426620APEX_matrix, targetP signal, mito protein domain, induction, coexpression++, MS/MSMatrixnonecerebellum, brainstem, largeintestine, testis
GLDCglycine ­decarboxylaseGCE, GCSP, HYGN1, P23378, gene:273120literature, APEX_matrix, targetP signal, yeast mito homolog, mito protein domain+, coexpression, MS/MS++MatrixMetabolism > Amino acid metabolism > Glycine metabolism;
Metabolism > Amino acid metabolism > Glycine cleavage system
cerebrum, cerebellum, kidney, liver, adipose, placenta
MPC1Lmitochondrial ­pyruvate ­carrier ­1 ­likeSLC54A3, P0DKB6, gene:34741120targetP signal, yeast mito homolog+, mito protein domain+, MS/MS++MIMMetabolism > Carbohydrate metabolism > Gluconeogenesis;
Metabolism > Carbohydrate metabolism > Pyruvate metabolism;
Metabolism > Carbohydrate metabolism > TCA-associated;
Small molecule transport
testis
THEM4thioesterase ­superfamily ­member ­4CTMP, Q5T1C6, gene:11714520literature, APEX_matrix, targetP signal+, yeast mito homolog, mito protein domain, induction, MS/MS++MIMMetabolism > Lipid metabolismcerebrum, cerebellum, brainstem, spinalcord, kidney, liver, adipose, smallintestine, largeintestine, placenta, testis
SSBP1single ­stranded ­DNA ­binding ­protein ­1Mt-SSB, SOSS-B1, SSBP, mtSSB, Q04837, gene:674219literature, APEX_matrix, targetP signal, yeast mito homolog++, Rickettsial homolog, mito protein domain, MS/MSMatrixMitochondrial central dogma > mtDNA maintenance > mtDNA replication;
Mitochondrial central dogma > mtDNA maintenance > mtDNA nucleoid
all 14
MRPL27mitochondrial ­ribosomal ­protein ­L27L27mt, Q9P0M9, gene:5126419literature, APEX_matrix, targetP signal, yeast mito homolog++, Rickettsial homolog, mito protein domain, MS/MSMatrixMitochondrial central dogma > Translation > Mitochondrial ribosomeall 14
HSCBHscB ­mitochondrial ­iron-sulfur ­cluster ­cochaperoneDNAJC20, HSC20, JAC1, Q8IWL3, gene:15027419APEX_matrix, GFP, targetP signal, yeast mito homolog++, Rickettsial homolog, mito protein domain, MS/MSMatrixMetabolism > Metals and cofactors > Fe-S cluster biosynthesiscerebrum, cerebellum, brainstem, spinalcord, skeletalmuscle, adipose, largeintestine, stomach, placenta, testis
MRPS10mitochondrial ­ribosomal ­protein ­S10MRP-S10, PNAS-122, P82664, gene:5517319literature, APEX_matrix, targetP signal, yeast mito homolog++, Rickettsial homolog, mito protein domain, MS/MS++MatrixMitochondrial central dogma > Translation > Mitochondrial ribosomeall 14
AK4adenylate ­kinase ­4AK 4, AK3, AK3L1, AK3L2, P27144, gene:20519APEX_matrix, yeast mito homolog++, Rickettsial homolog, mito protein domain, MS/MS++MatrixMetabolism > Nucleotide metabolism > Nucleotide synthesis and processingall 14
ATAD3AATPase ­family ­AAA ­domain ­containing ­3AHAYOS, PHRINL, Q9NVI7, gene:5521019APEX_IMS, APEX_matrix, GFP, Rickettsial homolog, mito protein domain+, coexpression, MS/MS++MIMMitochondrial central dogma > mtDNA maintenance > mtDNA nucleoid;
Mitochondrial dynamics and surveillance > Organelle contact sites;
Mitochondrial dynamics and surveillance > Intramitochondrial membrane interactions
all 14
MRPL37mitochondrial ­ribosomal ­protein ­L37L2mt, L37mt, MRP-L2, MRP-L37, MRPL2, RPML2, Q9BZE1, gene:5125319literature, APEX_matrix, targetP signal, mito protein domain, induction, coexpression+, MS/MS++MatrixMitochondrial central dogma > Translation > Mitochondrial ribosomekidney, liver, heart, adipose, smallintestine, largeintestine, stomach, placenta, testis
PTGES2prostaglandin ­E ­synthase ­2C9orf15, GBF-1, GBF1, PGES2, mPGES-2, Q9H7Z7, gene:8014219literature, APEX_IMS, APEX_matrix, targetP signal, mito protein domain, induction, coexpression+, MS/MS++MIMMetabolism > Lipid metabolism > Eicosanoid metabolismall 14
TXNRD2thioredoxin ­reductase ­2GCCD5, SELZ, TR, TR-BETA, TR3, TRXR2, Q9NNW7, gene:1058719literature, APEX_matrix, targetP signal+, Rickettsial homolog, mito protein domain, coexpression, MS/MS++MatrixMetabolism > Detoxification > ROS and glutathione metabolism;
Metabolism > Detoxification > Selenoproteins
cerebrum, spinalcord, kidney, liver, heart, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
ACSM5acyl-CoA ­synthetase ­medium ­chain ­family ­member ­5Q6NUN0, gene:5498819literature, targetP signal+, Rickettsial homolog, mito protein domain, coexpression, MS/MS++MatrixMetabolism > Lipid metabolism > Fatty acid oxidation;
Metabolism > Detoxification > Xenobiotic metabolism
kidney, liver, adipose, smallintestine, largeintestine
CISD1CDGSH ­iron ­sulfur ­domain ­1C10orf70, MDS029, ZCD1, mitoNEET, Q9NZ45, gene:5584719literature, mito protein domain, induction, coexpression++, MS/MS++MOMMetabolism > Metals and cofactors > Fe-S cluster biosynthesis;
Metabolism > Metals and cofactors > Fe-S-containing proteins
all 14
MRPL41mitochondrial ­ribosomal ­protein ­L41BMRP, MRP-L27, MRPL27, PIG3, RPML27, Q8IXM3, gene:6497519literature, APEX_matrix, yeast mito homolog++, mito protein domain, induction, MS/MS++MatrixMitochondrial central dogma > Translation > Mitochondrial ribosomeall 14
MTARC2mitochondrial ­amidoxime ­reducing ­component ­2MARC2, MOSC2, Q969Z3, gene:5499619literature, targetP signal, mito protein domain+, induction, coexpression, MS/MS++MOMMetabolism > Metals and cofactors > Molybdenum cofactor synthesis and proteins;
Metabolism > Detoxification > Xenobiotic metabolism;
Metabolism > Detoxification > Amidoxime reducing complex
all 14
MRPS6mitochondrial ­ribosomal ­protein ­S6C21orf101, MRP-S6, RPMS6, S6mt, P82932, gene:6496819literature, APEX_matrix, yeast mito homolog++, Rickettsial homolog, mito protein domain, MS/MS++MatrixMitochondrial central dogma > Translation > Mitochondrial ribosomeall 14
MRPL23mitochondrial ­ribosomal ­protein ­L23L23MRP, RPL23, RPL23L, Q16540, gene:615019literature, APEX_matrix, targetP signal, yeast mito homolog++, mito protein domain, MS/MS++MatrixMitochondrial central dogma > Translation > Mitochondrial ribosomeall 14
SQORsulfide ­quinone ­oxidoreductaseCGI-44, PRO1975, SQR, SQRDL, Q9Y6N5, gene:5847219literature, targetP signal, yeast mito homolog++, mito protein domain, MS/MS++MIMMetabolism > Electron carriers > Q-linked reactions, other;
Metabolism > Sulfur metabolism
spinalcord, kidney, liver, heart, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
SCO1synthesis ­of ­cytochrome ­C ­oxidase ­1SCOD1, O75880, gene:634119literature, APEX_IMS, APEX_matrix, targetP signal, yeast mito homolog+, Rickettsial homolog, mito protein domain, MS/MS++MIMOXPHOS > Complex IV > CIV assembly factors;
Metabolism > Metals and cofactors > Copper metabolism;
OXPHOS > OXPHOS assembly factors
cerebrum, cerebellum, brainstem, spinalcord, liver, heart, adipose, smallintestine, largeintestine, stomach, placenta, testis
GCATglycine ­C-acetyltransferaseKBL, O75600, gene:2346419literature, targetP signal, yeast mito homolog+, Rickettsial homolog, mito protein domain, MS/MS++unknownMetabolism > Amino acid metabolism > Glycine metabolismbrainstem, spinalcord, kidney, liver, adipose, smallintestine, largeintestine, stomach, placenta, testis
MTHFD1Lmethylenetetrahydrofolate ­dehydrogenase ­(NADP+ ­dependent) ­1 ­likeFTHFSDC1, MTC1THFS, dJ292B18.2, Q6UB35, gene:2590219literature, APEX_matrix, targetP signal, yeast mito homolog+, Rickettsial homolog, mito protein domain, MS/MS++MatrixMetabolism > Vitamin metabolism > Folate and 1-C metabolismcerebrum, cerebellum, brainstem, spinalcord, kidney, liver, adipose, smallintestine, largeintestine, stomach, placenta, testis
ECI2enoyl-CoA ­delta ­isomerase ­2ACBD2, DRS-1, DRS1, HCA88, PECI, dJ1013A10.3, O75521, gene:1045519literature, APEX_matrix, targetP signal+, mito protein domain, coexpression+, MS/MS++MatrixMetabolism > Lipid metabolism > Fatty acid oxidationall 14
UQCRBubiquinol-cytochrome ­c ­reductase ­binding ­proteinMC3DN3, QCR7, QP-C, QPC, UQBC, UQBP, UQCR6, UQPC, P14927, gene:738119literature, APEX_matrix, yeast mito homolog++, mito protein domain+, MS/MS++MIMOXPHOS > Complex III > CIII subunits;
OXPHOS > OXPHOS subunits
all 14
ATP5PDATP ­synthase ­peripheral ­stalk ­subunit ­dAPT5H, ATP5H, ATPQ, O75947, gene:1047619literature, APEX_matrix, yeast mito homolog++, mito protein domain+, MS/MS++MIMOXPHOS > Complex V > CV subunits;
OXPHOS > OXPHOS subunits
all 14
ATP5MGATP ­synthase ­membrane ­subunit ­gATP5JG, ATP5L, O75964, gene:1063219literature, APEX_matrix, yeast mito homolog++, mito protein domain+, MS/MS++MIMOXPHOS > Complex V > CV subunits;
OXPHOS > OXPHOS subunits;
Mitochondrial dynamics and surveillance > Cristae formation
all 14
CCDC58coiled-coil ­domain ­containing ­58Q4VC31, gene:13107619APEX_IMS, yeast mito homolog++, mito protein domain+, MS/MS++IMSnoneall 14
MCCC2methylcrotonoyl-CoA ­carboxylase ­2MCCB, Q9HCC0, gene:6408719literature, targetP signal, Rickettsial homolog, mito protein domain, coexpression+, MS/MS++MatrixMetabolism > Amino acid metabolism > BCAA metabolismall 14
MCEEmethylmalonyl-CoA ­epimeraseGLOD2, MCE, Q96PE7, gene:8469319targetP signal, induction, coexpression++, MS/MS++MatrixMetabolism > Carbohydrate metabolism > Propanoate metabolism;
Metabolism > Lipid metabolism > Fatty acid oxidation
all 14
MOCS1molybdenum ­cofactor ­synthesis ­1MIG11, MOCOD, MOCS1A, MOCS1B, Q9NZB8, gene:433719APEX_matrix, targetP signal+, Rickettsial homolog, mito protein domain+, MS/MS+MatrixMetabolism > Metals and cofactors > Molybdenum cofactor synthesis and proteinscerebrum, liver, adipose, smallintestine, largeintestine, stomach, placenta
MMUTmethylmalonyl-CoA ­mutaseMCM, MUT, P22033, gene:459419literature, APEX_matrix, targetP signal, mito protein domain+, coexpression, MS/MS++MatrixMetabolism > Carbohydrate metabolism > Propanoate metabolism;
Metabolism > Lipid metabolism > Fatty acid oxidation;
Metabolism > Vitamin metabolism > Vitamin B12 metabolism
all 14
SLC25A51solute ­carrier ­family ­25 ­member ­51CG7943, MCART1, Q9H1U9, gene:9201419GFP, yeast mito homolog+, mito protein domain+, coexpression, MS/MS++MIMMetabolism > Metals and cofactors > NAD biosynthesis and metabolism;
Small molecule transport > SLC25A family
cerebrum, cerebellum, brainstem, spinalcord, kidney, liver, heart, adipose, smallintestine, largeintestine, stomach, placenta, testis
SPG7SPG7 ­matrix ­AAA ­peptidase ­subunit, ­parapleginCAR, CMAR, PGN, SPG5C, Q9UQ90, gene:668719literature, targetP signal, Rickettsial homolog, mito protein domain+, coexpression+, MS/MSMIMProtein import, sorting and homeostasis > Protein homeostasis > Proteasescerebrum, cerebellum, brainstem, spinalcord, kidney, liver, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
ATP5MC3ATP ­synthase ­membrane ­subunit ­c ­locus ­3ATP5G3, P3, P48201, gene:51819literature, targetP signal, yeast mito homolog+, Rickettsial homolog, mito protein domain, coexpression++MIMOXPHOS > Complex V > CV subunits;
OXPHOS > OXPHOS subunits
none
ATAD1ATPase ­family ­AAA ­domain ­containing ­1AFDC1, FNP001, HKPX4, Msp1, THORASE, Q8NBU5, gene:8489619yeast mito homolog++, Rickettsial homolog, mito protein domain, coexpression, MS/MS++MOMProtein import, sorting and homeostasis > Protein homeostasis > Proteasescerebrum, cerebellum, brainstem, spinalcord, kidney, heart, skeletalmuscle, adipose, largeintestine, stomach, placenta, testis
OXNAD1oxidoreductase ­NAD ­binding ­domain ­containing ­1Q96HP4, gene:9210619APEX_matrix, GFP, mito protein domain, coexpression++, MS/MS++Matrixnonecerebrum, spinalcord, liver, heart, adipose, smallintestine, largeintestine, stomach, placenta, testis
MTG2mitochondrial ­ribosome ­associated ­GTPase ­2GTPBP5, ObgH1, dJ1005F21.2, Q9H4K7, gene:2616419literature, APEX_matrix, targetP signal+, yeast mito homolog+, Rickettsial homolog, mito protein domain, coexpressionMatrixMitochondrial central dogma > Translation > Mitochondrial ribosome assemblylargeintestine, placenta
DARS2aspartyl-tRNA ­synthetase ­2, ­mitochondrialASPRS, LBSL, MT-ASPRS, mtAspRS, Q6PI48, gene:5515719APEX_matrix, targetP signal, yeast mito homolog++, Rickettsial homolog, mito protein domain, coexpression, MS/MSMatrixMitochondrial central dogma > Translation > mt-tRNA synthetasescerebrum, cerebellum, brainstem, spinalcord, kidney, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
MRPL51mitochondrial ­ribosomal ­protein ­L51CDA09, HSPC241, MRP64, bMRP64, Q4U2R6, gene:5125819literature, APEX_matrix, targetP signal, mito protein domain+, coexpression, MS/MS++MatrixMitochondrial central dogma > Translation > Mitochondrial ribosomecerebrum, cerebellum, spinalcord, kidney, liver, heart, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
PCpyruvate ­carboxylasePCB, P11498, gene:509119literature, targetP signal, Rickettsial homolog, mito protein domain, coexpression, MS/MS++MatrixMetabolism > Carbohydrate metabolism > Gluconeogenesis;
Metabolism > Carbohydrate metabolism > TCA-associated;
Metabolism > Vitamin metabolism > Biotin utilizing proteins
all 14
CLYBLcitramalyl-CoA ­lyaseCLB, Q8N0X4, gene:17142519APEX_matrix, targetP signal+, induction, coexpression+, MS/MS++MatrixMetabolism > Carbohydrate metabolism > Itaconate metabolismall 14
PNPLA8patatin ­like ­phospholipase ­domain ­containing ­8IPLA2-2, IPLA2G, MMLA, PNPLA-gamma, iPLA2gamma, Q9NP80, gene:5064019APEX_matrix, Rickettsial homolog, induction, coexpression++, MS/MS+MIMMetabolism > Lipid metabolism > Cardiolipin synthesis;
Metabolism > Lipid metabolism > Phospholipid metabolism
cerebrum, cerebellum, brainstem, spinalcord, liver, adipose, largeintestine, testis
MLYCDmalonyl-CoA ­decarboxylaseMCD, O95822, gene:2341719literature, APEX_matrix, targetP signal+, mito protein domain, coexpression++, MS/MSMatrixMetabolism > Lipid metabolismcerebrum, cerebellum, brainstem, spinalcord, kidney, liver, heart, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta
PYURFPIGY ­upstream ­reading ­framePREY, Q96I23, gene:10099693919targetP signal+, induction, coexpression++, MS/MSunknownnonecerebrum, cerebellum, brainstem, spinalcord, liver, heart, skeletalmuscle, adipose, largeintestine, stomach, placenta
TIMM17Btranslocase ­of ­inner ­mitochondrial ­membrane ­17BDXS9822, JM3, TIM17B, O60830, gene:1024519literature, APEX_IMS, yeast mito homolog+, mito protein domain+, coexpression, MS/MS+MIMProtein import, sorting and homeostasis > Protein import and sorting > TIM23 presequence pathwayall 14
MRPL32mitochondrial ­ribosomal ­protein ­L32HSPC283, L32mt, MRP-L32, bMRP-59b, Q9BYC8, gene:6498319literature, APEX_matrix, yeast mito homolog++, Rickettsial homolog, mito protein domain, coexpression, MS/MSMatrixMitochondrial central dogma > Translation > Mitochondrial ribosomecerebrum, brainstem, spinalcord, liver, heart, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
SLC25A26solute ­carrier ­family ­25 ­member ­26COXPD28, SAMC, Q70HW3, gene:11528619literature, yeast mito homolog++, mito protein domain+, coexpression, MS/MSMIMSmall molecule transport > SLC25A familycerebrum, cerebellum, adipose, largeintestine, placenta, testis
PRODH2proline ­dehydrogenase ­2HSPOX1, HYPDH, Q9UF12, gene:5851019targetP signal, yeast mito homolog+, mito protein domain+, MS/MS+MIMMetabolism > Amino acid metabolism > Glyoxylate metabolism;
Metabolism > Electron carriers > Q-linked reactions, other
kidney, liver
NIT2nitrilase ­family ­member ­2HEL-S-8a, Q9NQR4, gene:5695419Rickettsial homolog, mito protein domain, coexpression, MS/MS++unknownMetabolism > Detoxificationcerebellum, spinalcord, kidney, liver, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
FDX2ferredoxin ­2FDX1L, MEOAL, Q6P4F2, gene:11281218APEX_matrix, targetP signal, yeast mito homolog+, Rickettsial homolog, mito protein domain, coexpression, MS/MSMatrixMetabolism > Metals and cofactors > Heme synthesis and processing;
Metabolism > Metals and cofactors > Fe-S cluster biosynthesis;
Metabolism > Metals and cofactors > Fe-S-containing proteins
cerebrum, cerebellum, brainstem, spinalcord, liver, skeletalmuscle, adipose, largeintestine, stomach, placenta, testis
ME2malic ­enzyme ­2ODS1, P23368, gene:420018literature, APEX_matrix, targetP signal, yeast mito homolog+, Rickettsial homolog, mito protein domain, MS/MS++MatrixMetabolism > Carbohydrate metabolism > TCA-associatedcerebrum, cerebellum, brainstem, spinalcord, kidney, heart, adipose, smallintestine, largeintestine, stomach, placenta, testis
TUFMTu ­translation ­elongation ­factor, ­mitochondrialCOXPD4, EF-TuMT, EFTU, P43, P49411, gene:728418literature, APEX_matrix, targetP signal, yeast mito homolog+, Rickettsial homolog, mito protein domain, MS/MS++MatrixMitochondrial central dogma > Translation > Translation factorsall 14
COX6A2cytochrome ­c ­oxidase ­subunit ­6A2COX6AH, COXVIAH, Q02221, gene:133918literature, targetP signal, yeast mito homolog+, mito protein domain+, induction, coexpressionMIMOXPHOS > Complex IV > CIV subunits;
OXPHOS > OXPHOS subunits
heart, skeletalmuscle
SLC30A9solute ­carrier ­family ­30 ­member ­9BILAPES, C4orf1, GAC63, HUEL, ZNT9, Q6PML9, gene:1046318APEX_matrix, targetP signal+, yeast mito homolog++, mito protein domain, induction, MS/MSMIMSmall molecule transportcerebrum, cerebellum, brainstem, spinalcord, adipose, largeintestine, stomach, placenta, testis
TAMM41TAM41 ­mitochondrial ­translocator ­assembly ­and ­maintenance ­homologC3orf31, RAM41, TAM41, Q96BW9, gene:13200118GFP, yeast mito homolog++, mito protein domain+, MS/MS++MIMMetabolism > Lipid metabolism > Cardiolipin synthesis;
Metabolism > Lipid metabolism > Phospholipid metabolism
cerebrum, cerebellum, brainstem, spinalcord, heart, skeletalmuscle, adipose, largeintestine, stomach, placenta, testis
TIMM23translocase ­of ­inner ­mitochondrial ­membrane ­23TIM23, O14925, gene:10028793218literature, APEX_IMS, yeast mito homolog++, mito protein domain+, MS/MS++MIMProtein import, sorting and homeostasis > Protein import and sorting > TIM23 presequence pathwayall 14
RMND1required ­for ­meiotic ­nuclear ­division ­1 ­homologC6orf96, COXPD11, RMD1, bA351K16, bA351K16.3, Q9NWS8, gene:5500518literature, GFP, targetP signal, yeast mito homolog++, Rickettsial homolog, mito protein domain, MS/MSMIMMitochondrial central dogma > mtRNA metabolism > mtRNA granules;
Mitochondrial central dogma > Translation
cerebrum, cerebellum, brainstem, spinalcord, adipose, smallintestine, largeintestine, stomach, placenta, testis
MFN2mitofusin ­2CMT2A, CMT2A2, CMT2A2A, CMT2A2B, CPRP1, HMSN6A, HSG, MARF, O95140, gene:992718literature, mito protein domain+, induction, coexpression, MS/MS+MOMMitochondrial dynamics and surveillance > Fusion;
Mitochondrial dynamics and surveillance > Organelle contact sites
cerebrum, cerebellum, brainstem, spinalcord, liver, adipose, largeintestine, stomach, placenta, testis
DNLZDNL-type ­zinc ­fingerC9orf151, HEP, HEP1, TIMM15, ZIM17, bA413M3.2, Q5SXM8, gene:72848918APEX_matrix, targetP signal, yeast mito homolog++, mito protein domain+, MS/MSMatrixProtein import, sorting and homeostasis > Protein homeostasis > Chaperonescerebrum, cerebellum, brainstem, heart, adipose, smallintestine, largeintestine, stomach, placenta
CHCHD4coiled-coil-helix-coiled-coil-helix ­domain ­containing ­4MIA40, TIMM40, Q8N4Q1, gene:13147418literature, APEX_IMS, yeast mito homolog++, mito protein domain, induction, coexpression, MS/MS+IMSProtein import, sorting and homeostasis > Protein import and sorting > MIA40cerebrum, cerebellum, brainstem, spinalcord, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
NDUFAB1NADH:ubiquinone ­oxidoreductase ­subunit ­AB1ACP, ACP1, FASN2A, SDAP, O14561, gene:470618literature, APEX_matrix, targetP signal+, yeast mito homolog, Rickettsial homolog, mito protein domain, MS/MS+MIMOXPHOS > Complex I > CI subunits;
Metabolism > Lipid metabolism > Type II fatty acid synthesis;
Metabolism > Metals and cofactors > Fe-S cluster biosynthesis;
OXPHOS > OXPHOS subunits
all 14
ACSM1acyl-CoA ­synthetase ­medium ­chain ­family ­member ­1BUCS1, MACS1, Q08AH1, gene:11628518literature, targetP signal, Rickettsial homolog, mito protein domain, coexpression, MS/MS++MatrixMetabolism > Lipid metabolism > Fatty acid oxidationspinalcord, kidney, liver, smallintestine
COA5cytochrome ­c ­oxidase ­assembly ­factor ­56330578E17Rik, C2orf64, CEMCOX3, Pet191, Q86WW8, gene:49375318literature, APEX_IMS, yeast mito homolog++, mito protein domain+, induction, MS/MSIMSOXPHOS > Complex IV > CIV assembly factors;
OXPHOS > OXPHOS assembly factors
adipose, placenta, testis
COQ2coenzyme ­Q2, ­polyprenyltransferaseCL640, COQ10D1, MSA1, PHB:PPT, Q96H96, gene:2723518literature, targetP signal, yeast mito homolog++, Rickettsial homolog, mito protein domain, coexpression+MIMMetabolism > Metals and cofactors > Coenzyme Q metabolismnone
PDSS2decaprenyl ­diphosphate ­synthase ­subunit ­2C6orf210, COQ10D3, COQ1B, DLP1, bA59I9.3, hDLP1, Q86YH6, gene:5710718literature, APEX_matrix, targetP signal, yeast mito homolog+, Rickettsial homolog, mito protein domain, coexpression+MIMMetabolism > Metals and cofactors > Coenzyme Q metabolismsmallintestine, largeintestine
EHHADHenoyl-CoA ­hydratase ­and ­3-hydroxyacyl ­CoA ­dehydrogenaseECHD, FRTS3, L-PBE, LBFP, LBP, MFE1, PBFE, Q08426, gene:196218yeast mito homolog+, Rickettsial homolog, mito protein domain, coexpression, MS/MS+unknownMetabolism > Lipid metabolismspinalcord, kidney, liver, adipose, smallintestine, largeintestine, placenta
NDUFS3NADH:ubiquinone ­oxidoreductase ­core ­subunit ­S3CI-30, MC1DN8, O75489, gene:472218literature, APEX_matrix, targetP signal+, Rickettsial homolog, mito protein domain, MS/MS++MIMOXPHOS > Complex I > CI subunits;
OXPHOS > OXPHOS subunits
all 14
ABCB10ATP ­binding ­cassette ­subfamily ­B ­member ­10EST20237, M-ABC2, MTABC2, Q9NRK6, gene:2345618literature, APEX_matrix, yeast mito homolog+, Rickettsial homolog, mito protein domain, induction, coexpression, MS/MS+MIMMetabolism > Metals and cofactors > Heme synthesis and processing;
Small molecule transport > ABC transporters
cerebellum, spinalcord, liver, heart, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
CKMT2creatine ­kinase, ­mitochondrial ­2SMTCK, P17540, gene:116018literature, targetP signal, mito protein domain, induction, coexpression, MS/MS++MIMMetabolism > Nucleotide metabolism > Creatine metabolismcerebrum, cerebellum, brainstem, spinalcord, heart, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta
MRPS25mitochondrial ­ribosomal ­protein ­S25MRP-S25, RPMS25, P82663, gene:6443218literature, APEX_matrix, targetP signal, mito protein domain, induction, coexpression, MS/MS++MatrixMitochondrial central dogma > Translation > Mitochondrial ribosomeall 14
ACAD9acyl-CoA ­dehydrogenase ­family ­member ­9MC1DN20, NPD002, Q9H845, gene:2897618literature, APEX_matrix, targetP signal+, mito protein domain+, MS/MS++MIMOXPHOS > Complex I > CI assembly factors;
OXPHOS > OXPHOS assembly factors
all 14
COX16cytochrome ­c ­oxidase ­assembly ­factor ­COX16C14orf112, HSPC203, hCOX16, Q9P0S2, gene:5124118APEX_IMS, GFP, yeast mito homolog++, mito protein domain+, MS/MS+MIMOXPHOS > Complex IV > CIV assembly factors;
OXPHOS > OXPHOS assembly factors
kidney, liver, adipose, largeintestine, placenta, testis
FAM210Afamily ­with ­sequence ­similarity ­210 ­member ­AC18orf19, HsT2329, Q96ND0, gene:12522818GFP, targetP signal, induction, coexpression++, MS/MSMIMnoneall 14
ACOT13acyl-CoA ­thioesterase ­13HT012, PNAS-27, THEM2, Q9NPJ3, gene:5585618mito protein domain, induction, coexpression, MS/MS++MatrixMetabolism > Lipid metabolismall 14
CPT1Acarnitine ­palmitoyltransferase ­1ACPT1, CPT1-L, L-CPT1, P50416, gene:137418literature, yeast mito homolog+, mito protein domain, induction, coexpression, MS/MS++MOMMetabolism > Lipid metabolism > Fatty acid oxidation;
Metabolism > Metals and cofactors > Carnitine synthesis and transport;
Metabolism > Metals and cofactors > Carnitine shuttle
all 14
DHRS4dehydrogenase/reductase ­4CR, NRDR, PHCR, PSCD, SCAD-SRL, SDR-SRL, SDR25C1, SDR25C2, Q9BTZ2, gene:1090118targetP signal, Rickettsial homolog, mito protein domain, coexpression, MS/MS++MatrixMetabolism > Vitamin metabolism > Vitamin A metabolismall 14
PRELID2PRELI ­domain ­containing ­2Q8N945, gene:15376818yeast mito homolog+, mito protein domain+, induction, coexpression, MS/MSIMSnonelargeintestine, stomach
CARS2cysteinyl-tRNA ­synthetase ­2, ­mitochondrialCOXPD27, cysRS, Q9HA77, gene:7958718APEX_matrix, targetP signal, Rickettsial homolog, mito protein domain, coexpression++, MS/MSMatrixMitochondrial central dogma > Translation > mt-tRNA synthetasescerebrum, heart, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
NIPSNAP2nipsnap ­homolog ­2GBAS, O75323, gene:263118literature, APEX_matrix, targetP signal+, induction, coexpression, MS/MS++MatrixMitochondrial dynamics and surveillance > Mitophagy;
Mitochondrial dynamics and surveillance > Autophagy
all 14
NDUFB7NADH:ubiquinone ­oxidoreductase ­subunit ­B7B18, CI-B18, P17568, gene:471318literature, APEX_IMS, APEX_matrix, induction, coexpression++, MS/MS++MIMOXPHOS > Complex I > CI subunits;
OXPHOS > OXPHOS subunits
all 14
MICOS13mitochondrial ­contact ­site ­and ­cristae ­organizing ­system ­subunit ­13C19orf70, MIC13, P117, QIL1, Q5XKP0, gene:12598818induction, coexpression++, MS/MS++MIMMitochondrial dynamics and surveillance > Cristae formation > MICOS complexall 14
GLRX2glutaredoxin ­2CGI-133, GRX2, Q9NS18, gene:5102217literature, targetP signal+, yeast mito homolog, Rickettsial homolog, mito protein domain, coexpression, MS/MSMatrixMetabolism > Metals and cofactors > Fe-S-containing proteins;
Metabolism > Vitamin metabolism > Vitamin C metabolism;
Metabolism > Detoxification > ROS and glutathione metabolism
cerebrum, cerebellum, brainstem, spinalcord, skeletalmuscle, largeintestine, placenta, testis
MRPS30mitochondrial ­ribosomal ­protein ­S30MRP-S30, PAP, PDCD9, S30mt, Q9NP92, gene:1088417literature, APEX_matrix, targetP signal, mito protein domain, induction, coexpression, MS/MS++MatrixMitochondrial central dogma > Translation > Mitochondrial ribosomecerebrum, cerebellum, spinalcord, kidney, heart, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
SCO2synthesis ­of ­cytochrome ­C ­oxidase ­2CEMCOX1, ECGF1, Gliostatin, MYP6, PD-ECGF, SCO1L, TP, TYMP, TdRPase, O43819, gene:999717literature, APEX_IMS, targetP signal+, yeast mito homolog+, Rickettsial homolog, mito protein domain, MS/MSMIMOXPHOS > Complex IV > CIV assembly factors;
Metabolism > Metals and cofactors > Copper metabolism;
OXPHOS > OXPHOS assembly factors
all 14
HSD17B10hydroxysteroid ­17-beta ­dehydrogenase ­1017b-HSD10, ABAD, CAMR, DUPXp11.22, ERAB, HADH2, HCD2, HSD10MD, MHBD, MRPP2, MRX17, MRX31, MRXS10, SCHAD, SDR5C1, Q99714, gene:302817literature, APEX_matrix, Rickettsial homolog, mito protein domain, induction, coexpression, MS/MS++MatrixMitochondrial central dogma > mtRNA metabolism > mtRNA granules;
Mitochondrial central dogma > mtRNA metabolism > Polycistronic mtRNA processing;
Mitochondrial central dogma > mtRNA metabolism > mt-tRNA modifications;
Metabolism > Lipid metabolism > Fatty acid oxidation;
Metabolism > Lipid metabolism > Cholesterol, bile acid, steroid synthesis;
Metabolism > Amino acid metabolism > BCAA metabolism
all 14
NARS2asparaginyl-tRNA ­synthetase ­2, ­mitochondrialDFNB94, SLM5, asnRS, Q96I59, gene:7973117GFP, targetP signal, yeast mito homolog++, Rickettsial homolog, mito protein domain, coexpressionMatrixMitochondrial central dogma > Translation > mt-tRNA synthetasesspinalcord, adipose, largeintestine, stomach, placenta
RPUSD3RNA ­pseudouridine ­synthase ­D3Q6P087, gene:28536717APEX_matrix, targetP signal, yeast mito homolog++, Rickettsial homolog, mito protein domain, coexpressionMatrixMitochondrial central dogma > mtRNA metabolism > mtRNA granules;
Mitochondrial central dogma > mtRNA metabolism > mt-rRNA modifications
largeintestine
SUOXsulfite ­oxidaseP51687, gene:682117APEX_IMS, targetP signal, mito protein domain+, coexpression, MS/MS++IMSMetabolism > Metals and cofactors > Molybdenum cofactor synthesis and proteins;
Metabolism > Sulfur metabolism
cerebrum, brainstem, spinalcord, kidney, liver, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
SARDHsarcosine ­dehydrogenaseBPR-2, DMGDHL1, SAR, SARD, SDH, Q9UL12, gene:175717literature, targetP signal+, mito protein domain, coexpression, MS/MS++MatrixMetabolism > Amino acid metabolism > Glycine metabolism;
Metabolism > Vitamin metabolism > Choline and betaine metabolism;
Metabolism > Vitamin metabolism > Folate and 1-C metabolism
cerebrum, cerebellum, brainstem, spinalcord, kidney, liver, adipose, smallintestine, largeintestine, stomach, placenta, testis
RIDAreactive ­intermediate ­imine ­deaminase ­A ­homologHRSP12, P14.5, PSP, UK114, hp14.5, P52758, gene:1024717APEX_matrix, yeast mito homolog++, mito protein domain, coexpression, MS/MS+Matrixnonecerebrum, cerebellum, brainstem, spinalcord, kidney, liver, heart, adipose, smallintestine, largeintestine, stomach, placenta, testis
COX8Acytochrome ­c ­oxidase ­subunit ­8ACOX, COX8, COX8-2, COX8L, VIII, VIII-L, P10176, gene:135117literature, targetP signal, mito protein domain+, coexpression+, MS/MSMIMOXPHOS > Complex IV > CIV subunits;
OXPHOS > OXPHOS subunits
stomach, placenta, testis
SPRsepiapterin ­reductaseSDR38C1, P35270, gene:669717Rickettsial homolog, mito protein domain, induction, coexpression, MS/MS++unknownMetabolism > Metals and cofactors > Tetrahydobiopterin synthesiscerebrum, cerebellum, brainstem, spinalcord, kidney, liver, heart, skeletalmuscle, adipose, largeintestine, stomach
MPV17mitochondrial ­inner ­membrane ­protein ­MPV17CMT2EE, MTDPS6, SYM1, P39210, gene:435817literature, yeast mito homolog+, mito protein domain, coexpression, MS/MS++MIMSmall molecule transportall 14
ZADH2zinc ­binding ­alcohol ­dehydrogenase ­domain ­containing ­2PRG-3, Q8N4Q0, gene:28427317mito protein domain, induction, coexpression+, MS/MS++unknownMetabolism > Lipid metabolism > Eicosanoid metabolismall 14
MRPL18mitochondrial ­ribosomal ­protein ­L18HSPC071, L18mt, MRP-L18, Q9H0U6, gene:2907417literature, APEX_matrix, targetP signal+, Rickettsial homolog, mito protein domain, coexpression, MS/MSMatrixMitochondrial central dogma > Translation > Mitochondrial ribosomeall 14
ALDH1B1aldehyde ­dehydrogenase ­1 ­family ­member ­B1ALDH5, ALDHX, P30837, gene:21917literature, APEX_matrix, targetP signal, yeast mito homolog+, mito protein domain, MS/MS++MatrixMetabolism > Detoxification > Xenobiotic metabolismall 14
CHCHD1coiled-coil-helix-coiled-coil-helix ­domain ­containing ­1C10orf34, C2360, MRP-S37, Q96BP2, gene:11848717GFP, targetP signal, yeast mito homolog+, mito protein domain, coexpression+, MS/MSMIMMitochondrial central dogma > Translation > Mitochondrial ribosomecerebrum, cerebellum, spinalcord, kidney, liver, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
TIMM8Atranslocase ­of ­inner ­mitochondrial ­membrane ­8ADDP, DDP1, DFN1, MTS, TIM8, O60220, gene:167817literature, yeast mito homolog+, mito protein domain+, MS/MS++MIMProtein import, sorting and homeostasis > Protein homeostasis > Chaperones;
Protein import, sorting and homeostasis > Protein import and sorting
all 14
VDAC3voltage ­dependent ­anion ­channel ­3HD-VDAC3, VDAC-3, Q9Y277, gene:741917literature, yeast mito homolog+, mito protein domain+, MS/MS++MOMSignaling > Calcium homeostasis > Mitochondrial permeability transition pore;
Small molecule transport
all 14
SLC25A24solute ­carrier ­family ­25 ­member ­24APC1, SCAMC-1, SCAMC1, Q6NUK1, gene:2995717literature, APEX_IMS, yeast mito homolog+, mito protein domain+, MS/MS++MIMMetabolism > Nucleotide metabolism > Nucleotide import;
Signaling > Calcium homeostasis > EF hand proteins;
Small molecule transport > SLC25A family
adipose, smallintestine, largeintestine, stomach, placenta, testis
MPC1mitochondrial ­pyruvate ­carrier ­1BRP44L, CGI-129, MPYCD, SLC54A1, Q9Y5U8, gene:5166017GFP, yeast mito homolog+, mito protein domain+, MS/MS++MIMMetabolism > Carbohydrate metabolism > Gluconeogenesis;
Metabolism > Carbohydrate metabolism > Pyruvate metabolism;
Metabolism > Carbohydrate metabolism > TCA-associated;
Small molecule transport
all 14
SLC25A22solute ­carrier ­family ­25 ­member ­22EIEE3, GC-1, GC1, NET44, Q9H936, gene:7975117literature, yeast mito homolog+, mito protein domain+, MS/MS++MIMSmall molecule transport > SLC25A familycerebrum, cerebellum, brainstem, spinalcord, kidney, liver, heart, adipose, smallintestine, largeintestine, stomach, placenta, testis
SLC25A31solute ­carrier ­family ­25 ­member ­31AAC4, ANT 4, ANT4, SFEC35kDa, Q9H0C2, gene:8344717literature, yeast mito homolog+, mito protein domain+, MS/MS++MIMMetabolism > Nucleotide metabolism > Nucleotide import;
Signaling > Calcium homeostasis > Mitochondrial permeability transition pore;
Small molecule transport > SLC25A family
all 14
SLC25A18solute ­carrier ­family ­25 ­member ­18GC2, Q9H1K4, gene:8373317literature, yeast mito homolog+, mito protein domain+, MS/MS++MIMSmall molecule transport > SLC25A familycerebrum, cerebellum, brainstem, spinalcord, kidney, liver, adipose, smallintestine, largeintestine, stomach, placenta, testis
TRNT1tRNA ­nucleotidyl ­transferase ­1CCA1, CGI-47, MtCCA, RPEM, SIFD, Q96Q11, gene:5109517literature, targetP signal, yeast mito homolog, Rickettsial homolog, mito protein domain, induction, MS/MSMatrixMitochondrial central dogma > mtRNA metabolism > mt-tRNA modificationsspinalcord, adipose, placenta, testis
IDEinsulin ­degrading ­enzymeINSULYSIN, P14735, gene:341617literature, APEX_matrix, targetP signal, Rickettsial homolog, mito protein domain+, coexpression, MS/MS+MatrixProtein import, sorting and homeostasis > Protein homeostasis > Proteasescerebrum, cerebellum, brainstem, kidney, liver, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
GSTK1glutathione ­S-transferase ­kappa ­1GST, GST 13-13, GST13, GST13-13, GSTK1-1, hGSTK1, Q9Y2Q3, gene:37315617literature, mito protein domain+, coexpression+, MS/MS++MatrixMetabolism > Detoxification > Xenobiotic metabolismall 14
VARS2valyl-tRNA ­synthetase ­2, ­mitochondrialCOXPD20, VALRS, VARS2L, VARSL, Q5ST30, gene:5717617APEX_matrix, targetP signal, Rickettsial homolog, mito protein domain, coexpression, MS/MS++MatrixMitochondrial central dogma > Translation > mt-tRNA synthetasescerebrum, cerebellum, brainstem, spinalcord, kidney, liver, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
HSPE1heat ­shock ­protein ­family ­E ­(Hsp10) ­member ­1CPN10, EPF, GROES, HSP10, P61604, gene:333617literature, APEX_IMS, APEX_matrix, yeast mito homolog++, Rickettsial homolog, mito protein domain, MS/MS++MatrixProtein import, sorting and homeostasis > Protein homeostasis > Chaperonesall 14
MRPL54mitochondrial ­ribosomal ­protein ­L54L54mt, MRP-L54, Q6P161, gene:11654117literature, APEX_matrix, yeast mito homolog++, mito protein domain, coexpression, MS/MS+MatrixMitochondrial central dogma > Translation > Mitochondrial ribosomecerebrum, cerebellum, brainstem, spinalcord, kidney, liver, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
MRPL38mitochondrial ­ribosomal ­protein ­L38HSPC262, L38MT, MRP-L3, MRP-L38, RPML3, Q96DV4, gene:6497817APEX_matrix, targetP signal, mito protein domain, coexpression++, MS/MS+MatrixMitochondrial central dogma > Translation > Mitochondrial ribosomecerebrum, spinalcord, heart, smallintestine, largeintestine, stomach, placenta, testis
MRPL58mitochondrial ­ribosomal ­protein ­L58DS-1, DS1, ICT1, MRP-L58, Q14197, gene:339617APEX_matrix, GFP, targetP signal+, mito protein domain, induction, coexpression, MS/MSMatrixMitochondrial central dogma > Translation > Mitochondrial ribosome;
Mitochondrial central dogma > Translation > Translation factors
all 14
MTCH1mitochondrial ­carrier ­1CGI-64, PIG60, PSAP, SLC25A49, Q9NZJ7, gene:2378717literature, yeast mito homolog+, mito protein domain+, MS/MS++MOMSmall molecule transport > SLC25A familycerebrum, cerebellum, brainstem, spinalcord, kidney, heart, adipose, smallintestine, largeintestine, stomach, placenta, testis
TRIAP1TP53 ­regulated ­inhibitor ­of ­apoptosis ­1HSPC132, MDM35, P53CSV, WF-1, O43715, gene:5149917literature, yeast mito homolog++, mito protein domain, induction, MS/MS++IMSMetabolism > Lipid metabolism > Cardiolipin synthesis;
Metabolism > Lipid metabolism > Phospholipid metabolism
adipose, stomach, placenta
PTCD3pentatricopeptide ­repeat ­domain ­3MRP-S39, Q96EY7, gene:5503717literature, APEX_matrix, targetP signal+, mito protein domain+, MS/MS++MatrixMitochondrial central dogma > Translation > Mitochondrial ribosome;
Mitochondrial central dogma > Translation > Mitochondrial ribosome assembly
all 14
COX10cytochrome ­c ­oxidase ­assembly ­factor ­heme ­A:farnesyltransferase ­COX10Q12887, gene:135217literature, yeast mito homolog, Rickettsial homolog, mito protein domain, induction, coexpression++MIMOXPHOS > Complex IV > CIV assembly factors;
Metabolism > Metals and cofactors > Heme synthesis and processing;
OXPHOS > OXPHOS assembly factors
none
AKAP1A-kinase ­anchoring ­protein ­1AKAP, AKAP121, AKAP149, AKAP84, D-AKAP1, PPP1R43, PRKA1, SAKAP84, TDRD17, Q92667, gene:816517literature, mito protein domain, induction, coexpression++, MS/MS+MOMSignaling > cAMP-PKA signalingcerebellum, skeletalmuscle, testis
MRM3mitochondrial ­rRNA ­methyltransferase ­3RMTL1, RNMTL1, Q9HC36, gene:5517817APEX_matrix, GFP, targetP signal+, mito protein domain+, induction, coexpressionMatrixMitochondrial central dogma > mtRNA metabolism > mt-rRNA modifications;
Mitochondrial central dogma > Translation > Mitochondrial ribosome assembly
none
AK2adenylate ­kinase ­2ADK2, P54819, gene:20417literature, APEX_IMS, Rickettsial homolog, mito protein domain, coexpression+, MS/MS++IMSMetabolism > Nucleotide metabolism > Nucleotide synthesis and processingall 14
TMEM70transmembrane ­protein ­70MC5DN2, Q9BUB7, gene:5496817literature, APEX_matrix, mito protein domain+, coexpression++, MS/MSMIMOXPHOS > Complex I > CI assembly factors;
OXPHOS > Complex V > CV assembly factors;
OXPHOS > OXPHOS assembly factors
cerebrum, cerebellum, brainstem, spinalcord, liver, heart, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
NOA1nitric ­oxide ­associated ­1C4orf14, MTG3, hAtNOS1, hNOA1, mAtNOS1, Q8NC60, gene:8427317literature, APEX_matrix, targetP signal+, yeast mito homolog++, mito protein domain, coexpressionMIMMitochondrial central dogma > mtRNA metabolism > mtRNA granules;
Mitochondrial central dogma > Translation > Mitochondrial ribosome assembly
adipose, largeintestine
MCUR1mitochondrial ­calcium ­uniporter ­regulator ­1C6orf79, CCDC90A, FMP32, Q96AQ8, gene:6393317APEX_matrix, yeast mito homolog+, mito protein domain+, induction, MS/MSMIMnonecerebrum, cerebellum, brainstem, spinalcord, kidney, heart, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta
TMEM14Ctransmembrane ­protein ­14CC6orf53, HSPC194, MSTP073, NET26, bA421M1.6, Q9P0S9, gene:5152217literature, mito protein domain+, induction, coexpression, MS/MS+MIMnoneall 14
NDUFC1NADH:ubiquinone ­oxidoreductase ­subunit ­C1KFYI, O43677, gene:471717literature, targetP signal+, mito protein domain+, coexpression++MIMOXPHOS > Complex I > CI subunits;
OXPHOS > OXPHOS subunits
none
MRPL35mitochondrial ­ribosomal ­protein ­L35L35mt, MRP-L35, Q9NZE8, gene:5131817literature, APEX_matrix, targetP signal, mito protein domain, induction, coexpression++MatrixMitochondrial central dogma > Translation > Mitochondrial ribosomelargeintestine, testis
NDUFA3NADH:ubiquinone ­oxidoreductase ­subunit ­A3B9, CI-B9, O95167, gene:469617literature, induction, coexpression++, MS/MS++MIMOXPHOS > Complex I > CI subunits;
OXPHOS > OXPHOS subunits
all 14
PDSS1decaprenyl ­diphosphate ­synthase ­subunit ­1COQ1, COQ10D2, COQ1A, DPS, SPS, TPRT, TPT, TPT 1, hDPS1, Q5T2R2, gene:2359017APEX_matrix, targetP signal, yeast mito homolog+, Rickettsial homolog, mito protein domain, coexpressionMIMMetabolism > Metals and cofactors > Coenzyme Q metabolismskeletalmuscle, largeintestine, placenta, testis
SLC25A16solute ­carrier ­family ­25 ­member ­16D10S105E, GDA, GDC, HGT.1, ML7, hML7, P16260, gene:803417literature, yeast mito homolog+, mito protein domain+, coexpression, MS/MSMIMSmall molecule transport > SLC25A familycerebrum, brainstem, spinalcord, liver, adipose, largeintestine, stomach, placenta, testis
FMC1formation ­of ­mitochondrial ­complex ­V ­assembly ­factor ­1 ­homologC7orf55, HSPC268, 0, gene:15479117literature, APEX_matrix, targetP signal, induction, coexpression++, MS/MSMatrixOXPHOS > Complex V > CV assembly factors;
OXPHOS > OXPHOS assembly factors
cerebrum, cerebellum, brainstem, spinalcord, kidney, liver, heart, adipose, smallintestine, largeintestine, stomach, placenta, testis
ACSM3acyl-CoA ­synthetase ­medium ­chain ­family ­member ­3SA, SAH, Q53FZ2, gene:629617literature, targetP signal+, Rickettsial homolog, mito protein domain, MS/MS++MatrixMetabolism > Lipid metabolism > Fatty acid oxidationkidney, liver, adipose, smallintestine, largeintestine, stomach
NDUFAF6NADH:ubiquinone ­oxidoreductase ­complex ­assembly ­factor ­6C8orf38, FRTS5, MC1DN17, Q330K2, gene:13768216literature, targetP signal, mito protein domain, induction, coexpression++, MS/MSMIMOXPHOS > Complex I > CI assembly factors;
OXPHOS > OXPHOS assembly factors
adipose, smallintestine, largeintestine, placenta, testis
UCP1uncoupling ­protein ­1SLC25A7, UCP, P25874, gene:735016literature, yeast mito homolog+, mito protein domain+, coexpression, MS/MSMIMSmall molecule transport > SLC25A familyadipose
SFXN1sideroflexin ­1SLC56A1, TCC, Q9H9B4, gene:9408116literature, mito protein domain+, coexpression, MS/MS++MIMMetabolism > Amino acid metabolism > Serine metabolism;
Metabolism > Vitamin metabolism > Folate and 1-C metabolism;
Small molecule transport > Sideroflexins
cerebrum, cerebellum, brainstem, spinalcord, kidney, liver, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
GLRX5glutaredoxin ­5C14orf87, FLB4739, GRX5, PR01238, PRO1238, PRSA, SIDBA3, SPAHGC, Q86SX6, gene:5121816literature, APEX_matrix, yeast mito homolog+, Rickettsial homolog, mito protein domain, coexpression, MS/MS+MatrixMetabolism > Metals and cofactors > Fe-S cluster biosynthesis;
Metabolism > Metals and cofactors > Fe-S-containing proteins
all 14
PTRH1peptidyl-tRNA ­hydrolase ­1 ­homologC9orf115, PTH1, Q86Y79, gene:13842816APEX_matrix, GFP, targetP signal, yeast mito homolog++, Rickettsial homolog, mito protein domain, MS/MSMatrixnonecerebrum, cerebellum, brainstem, largeintestine, placenta
MRPS11mitochondrial ­ribosomal ­protein ­S11HCC-2, MRP-S11, S11mt, P82912, gene:6496316literature, targetP signal, Rickettsial homolog, mito protein domain, induction, coexpression, MS/MSMatrixMitochondrial central dogma > Translation > Mitochondrial ribosomeall 14
XPNPEP3X-prolyl ­aminopeptidase ­3APP3, ICP55, NPHPL1, Q9NQH7, gene:6392916literature, APEX_matrix, yeast mito homolog, Rickettsial homolog, mito protein domain, coexpression, MS/MS+MatrixProtein import, sorting and homeostasis > Protein import and sorting > Preprotein cleavage;
Protein import, sorting and homeostasis > Protein homeostasis > Proteases
cerebrum, liver, adipose, smallintestine, largeintestine, stomach, placenta, testis
PDP1pyruvate ­dehyrogenase ­phosphatase ­catalytic ­subunit ­1PDH, PDP, PDPC, PPM2A, PPM2C, Q9P0J1, gene:5470416literature, APEX_matrix, targetP signal, yeast mito homolog+, mito protein domain, MS/MS++MIMMetabolism > Carbohydrate metabolism > Pyruvate metabolismcerebrum, cerebellum, spinalcord, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
ECI1enoyl-CoA ­delta ­isomerase ­1DCI, P42126, gene:163216literature, APEX_matrix, targetP signal, mito protein domain, coexpression+, MS/MS++MatrixMetabolism > Lipid metabolism > Fatty acid oxidationall 14
SLC25A21solute ­carrier ­family ­25 ­member ­21MTDPS18, ODC, ODC1, Q9BQT8, gene:8987416literature, yeast mito homolog+, mito protein domain+, MS/MS++MIMMetabolism > Amino acid metabolism;
Small molecule transport > SLC25A family
spinalcord, kidney, liver, testis
SLC25A29solute ­carrier ­family ­25 ­member ­29C14orf69, CACL, ORNT3, Q8N8R3, gene:12309616literature, yeast mito homolog+, mito protein domain+, MS/MS++MIMMetabolism > Amino acid metabolism > Lysine metabolism;
Small molecule transport > SLC25A family
placenta, testis
SLC25A45solute ­carrier ­family ­25 ­member ­45Q8N413, gene:28313016yeast mito homolog+, mito protein domain+, MS/MS++MIMSmall molecule transport > SLC25A familyliver, adipose, smallintestine, stomach, placenta, testis
SDHAF3succinate ­dehydrogenase ­complex ­assembly ­factor ­3ACN9, DC11, LYRM10, Sdh7, Q9NRP4, gene:5700116targetP signal, yeast mito homolog++, coexpression+, MS/MSMatrixOXPHOS > Complex II > CII assembly factors;
OXPHOS > OXPHOS assembly factors
adipose, testis
PRELID3BPRELI ­domain ­containing ­3BC20orf45, SLMO2, dJ543J19.5, Q9Y3B1, gene:5101216GFP, yeast mito homolog+, mito protein domain+, induction, coexpressionIMSMetabolism > Lipid metabolism > Phospholipid metabolismnone
SLC25A39solute ­carrier ­family ­25 ­member ­39CGI-69, CGI69, Q9BZJ4, gene:5162916literature, yeast mito homolog+, mito protein domain+, induction, coexpressionMIMSmall molecule transport > SLC25A familynone
NDUFB3NADH:ubiquinone ­oxidoreductase ­subunit ­B3B12, CI-B12, MC1DN25, O43676, gene:470916literature, APEX_IMS, APEX_matrix, mito protein domain+, coexpression+, MS/MS++MIMOXPHOS > Complex I > CI subunits;
OXPHOS > OXPHOS subunits
all 14
REXO2RNA ­exonuclease ­2CGI-114, REX2, RFN, SFN, Q9Y3B8, gene:2599616APEX_matrix, targetP signal, yeast mito homolog++, mito protein domain, MS/MS+MatrixMitochondrial central dogma > mtRNA metabolism > mtRNA stability and decayall 14
GATMglycine ­amidinotransferaseAGAT, AT, CCDS3, FRTS1, P50440, gene:262816literature, targetP signal, mito protein domain+, MS/MS++MatrixMetabolism > Nucleotide metabolism > Creatine metabolismspinalcord, kidney, liver, stomach, placenta, testis
TIMM10Btranslocase ­of ­inner ­mitochondrial ­membrane ­10BFXC1, TIM10B, Tim9b, Q9Y5J6, gene:2651516literature, APEX_IMS, yeast mito homolog+, mito protein domain+, MS/MS+MIMProtein import, sorting and homeostasis > Protein homeostasis > Chaperones;
Protein import, sorting and homeostasis > Protein import and sorting
cerebrum, cerebellum, brainstem, spinalcord, kidney, liver, heart, adipose, largeintestine, stomach, placenta, testis
SARS2seryl-tRNA ­synthetase ­2, ­mitochondrialSARS, SARSM, SERS, SYS, SerRS, SerRSmt, mtSerRS, Q9NP81, gene:5493816literature, APEX_matrix, targetP signal, yeast mito homolog++, Rickettsial homolog, mito protein domain, MS/MSMatrixMitochondrial central dogma > Translation > mt-tRNA synthetasesskeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
CHDHcholine ­dehydrogenaseQ8NE62, gene:5534916APEX_matrix, targetP signal+, mito protein domain, coexpression, MS/MS++MIMMetabolism > Amino acid metabolism > Glycine metabolism;
Metabolism > Vitamin metabolism > Choline and betaine metabolism;
Metabolism > Vitamin metabolism > Folate and 1-C metabolism;
Metabolism > Electron carriers > Q-linked reactions, other
spinalcord, kidney, liver, adipose, smallintestine, largeintestine, stomach, placenta, testis
MRPL57mitochondrial ­ribosomal ­protein ­L57MRP63, bMRP63, Q9BQC6, gene:7898816literature, APEX_matrix, targetP signal, mito protein domain+, coexpression, MS/MS+MatrixMitochondrial central dogma > Translation > Mitochondrial ribosomecerebrum, spinalcord, kidney, liver, adipose, smallintestine, largeintestine, placenta, testis
C8orf82chromosome ­8 ­open ­reading ­frame ­82Q6P1X6, gene:41491916APEX_matrix, targetP signal, coexpression+, MS/MS++Matrixnoneall 14
EARS2glutamyl-tRNA ­synthetase ­2, ­mitochondrialCOXPD12, MSE1, gluRS, mtGlnRS, Q5JPH6, gene:12445416literature, APEX_matrix, GFP, targetP signal, yeast mito homolog++, Rickettsial homolog, mito protein domain, coexpressionMatrixMitochondrial central dogma > Translation > mt-tRNA synthetasesadipose, largeintestine, placenta
GRHPRglyoxylate ­and ­hydroxypyruvate ­reductaseGLXR, GLYD, PH2, Q9UBQ7, gene:938016APEX_matrix, targetP signal, yeast mito homolog, mito protein domain, coexpression, MS/MS++MatrixMetabolism > Amino acid metabolism > Glyoxylate metabolismcerebrum, cerebellum, brainstem, spinalcord, kidney, liver, heart, skeletalmuscle, adipose, largeintestine, stomach, placenta, testis
MTFMTmitochondrial ­methionyl-tRNA ­formyltransferaseCOXPD15, FMT1, MC1DN27, Q96DP5, gene:12326316literature, APEX_matrix, targetP signal+, yeast mito homolog++, Rickettsial homolog, mito protein domain, coexpressionMatrixMitochondrial central dogma > Translation > fMet processing;
Metabolism > Vitamin metabolism > Folate and 1-C metabolism
none
PTCD2pentatricopeptide ­repeat ­domain ­2Q8WV60, gene:7981016literature, mito protein domain+, induction, coexpression, MS/MSMatrixMitochondrial central dogma > mtRNA metabolism > mtRNA granules;
Mitochondrial central dogma > mtRNA metabolism > Polycistronic mtRNA processing
smallintestine, largeintestine, placenta
ECHDC1ethylmalonyl-CoA ­decarboxylase ­1HEL-S-76, MMCD, dJ351K20.2, Q9NTX5, gene:5586216yeast mito homolog+, Rickettsial homolog, mito protein domain, coexpression, MS/MS+unknownMetabolism > Lipid metabolismbrainstem, spinalcord, liver, adipose, largeintestine, placenta, testis
HARS2histidyl-tRNA ­synthetase ­2, ­mitochondrialHARSL, HARSR, HO3, HisRS, PRLTS2, P49590, gene:2343816literature, APEX_matrix, GFP, targetP signal, Rickettsial homolog, mito protein domain, coexpression, MS/MS+MatrixMitochondrial central dogma > Translation > mt-tRNA synthetasescerebellum, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
NDUFA4NDUFA4 ­mitochondrial ­complex ­associatedCI-9k, CI-MLRQ, COXFA4, MLRQ, O00483, gene:469716literature, mito protein domain, coexpression++, MS/MS++MIMOXPHOS > Complex IV > CIV subunits;
OXPHOS > OXPHOS subunits
all 14
NDUFA13NADH:ubiquinone ­oxidoreductase ­subunit ­A13B16.6, CDA016, CGI-39, GRIM-19, GRIM19, MC1DN28, Q9P0J0, gene:5107916literature, APEX_IMS, mito protein domain, coexpression++, MS/MS++MIMOXPHOS > Complex I > CI subunits;
OXPHOS > OXPHOS subunits
all 14
NAXENAD(P)HX ­epimeraseAIBP, APOA1BP, PEBEL, YJEFN1, Q8NCW5, gene:12824016APEX_matrix, targetP signal, mito protein domain, coexpression, MS/MS++MatrixMetabolism > Metals and cofactors > NAD biosynthesis and metabolismall 14
TMEM11transmembrane ­protein ­11C17orf35, PM1, PMI, P17152, gene:883416GFP, targetP signal, mito protein domain+, induction, coexpression, MS/MSMIMMitochondrial dynamics and surveillanceall 14
SELENOOselenoprotein ­OSELO, Q9BVL4, gene:8364215targetP signal+, yeast mito homolog, mito protein domain+, coexpressionMatrixMetabolism > Detoxification > Selenoproteinsspinalcord, liver, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
NAGSN-acetylglutamate ­synthaseAGAS, ARGA, Q8N159, gene:16241715literature, targetP signal, yeast mito homolog++, mito protein domain+, MS/MSMatrixMetabolism > Amino acid metabolism > Urea cycleliver
HSD17B4hydroxysteroid ­17-beta ­dehydrogenase ­4DBP, MFE-2, MFP-2, MPF-2, PRLTS1, SDR8C1, P51659, gene:329515Rickettsial homolog, mito protein domain+, induction, coexpressionunknownMetabolism > Lipid metabolismkidney, liver, adipose, largeintestine, stomach, testis
NDUFAF7NADH:ubiquinone ­oxidoreductase ­complex ­assembly ­factor ­7C2orf56, MidA, PRO1853, Q7L592, gene:5547115APEX_matrix, GFP, targetP signal, Rickettsial homolog, mito protein domain+, coexpression, MS/MSMIMOXPHOS > Complex I > CI assembly factors;
OXPHOS > OXPHOS assembly factors
cerebrum, brainstem, spinalcord, kidney, adipose, largeintestine, stomach, placenta, testis
OATornithine ­aminotransferaseGACR, HOGA, OATASE, OKT, P04181, gene:494215literature, APEX_matrix, targetP signal+, mito protein domain, coexpression, MS/MS++MatrixMetabolism > Amino acid metabolism > Proline metabolismall 14
MRPL50mitochondrial ­ribosomal ­protein ­L50MRP-L50, Q8N5N7, gene:5453415literature, APEX_matrix, targetP signal, mito protein domain+, coexpression+, MS/MSMatrixMitochondrial central dogma > Translation > Mitochondrial ribosomecerebrum, cerebellum, brainstem, spinalcord, kidney, liver, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
COX7Bcytochrome ­c ­oxidase ­subunit ­7BAPLCC, LSDMCA2, P24311, gene:134915literature, targetP signal, mito protein domain+, induction, MS/MS+MIMOXPHOS > Complex IV > CIV subunits;
OXPHOS > OXPHOS subunits
cerebrum, cerebellum, brainstem, spinalcord, kidney, liver, heart, adipose, smallintestine, largeintestine, stomach, placenta
RARS2arginyl-tRNA ­synthetase ­2, ­mitochondrialArgRS, DALRD2, PCH6, PRO1992, RARSL, Q5T160, gene:5703815literature, APEX_matrix, GFP, Rickettsial homolog, mito protein domain, coexpression, MS/MS++MatrixMitochondrial central dogma > Translation > mt-tRNA synthetasesall 14
OXLD1oxidoreductase ­like ­domain ­containing ­1C17orf90, Q5BKU9, gene:33922915targetP signal, yeast mito homolog, mito protein domain+, coexpression+unknownnoneadipose, largeintestine, stomach
LACTB2lactamase ­beta ­2CGI-83, Q53H82, gene:5111015targetP signal, yeast mito homolog+, mito protein domain, induction, MS/MS+MatrixMitochondrial central dogma > mtRNA metabolism > mtRNA stability and decaykidney, liver, largeintestine, stomach
NDUFA11NADH:ubiquinone ­oxidoreductase ­subunit ­A11B14.7, CI-B14.7, MC1DN14, Q86Y39, gene:12632815literature, APEX_IMS, mito protein domain+, induction, MS/MS++MIMOXPHOS > Complex I > CI subunits;
OXPHOS > OXPHOS subunits
all 14
AURKAIP1aurora ­kinase ­A ­interacting ­protein ­1AIP, AKIP, MRP-S38, Q9NWT8, gene:5499815GFP, targetP signal, yeast mito homolog++, mito protein domain, coexpression+MatrixMitochondrial central dogma > Translation > Mitochondrial ribosomenone
CHCHD7coiled-coil-helix-coiled-coil-helix ­domain ­containing ­7COX23, Q9BUK0, gene:7914515yeast mito homolog, induction, coexpression, MS/MS+IMSMetabolism > Metals and cofactors > Copper metabolismcerebrum, smallintestine, placenta, testis
SHMT2serine ­hydroxymethyltransferase ­2GLYA, HEL-S-51e, SHMT, P34897, gene:647215literature, APEX_matrix, targetP signal, Rickettsial homolog, mito protein domain, coexpression, MS/MS++MatrixMetabolism > Amino acid metabolism > Serine metabolism;
Metabolism > Amino acid metabolism > Glycine metabolism;
Metabolism > Vitamin metabolism > Folate and 1-C metabolism
cerebrum, brainstem, spinalcord, kidney, liver, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
MTHFD2methylenetetrahydrofolate ­dehydrogenase ­(NADP+ ­dependent) ­2, ­methenyltetrahydrofolate ­cyclohydrolaseNMDMC, P13995, gene:1079715literature, APEX_matrix, targetP signal, yeast mito homolog+, Rickettsial homolog, mito protein domain, MS/MSMatrixMetabolism > Vitamin metabolism > Folate and 1-C metabolismspinalcord, placenta, testis
RMDN1regulator ­of ­microtubule ­dynamics ­1CGI-90, FAM82B, RMD-1, RMD1, Q96DB5, gene:5111515APEX_IMS, targetP signal+, induction, coexpression, MS/MSMOMnoneall 14
CPS1carbamoyl-phosphate ­synthase ­1CPSASE1, PHN, P31327, gene:137315literature, APEX_matrix, targetP signal, Rickettsial homolog, mito protein domain, MS/MS++MatrixMetabolism > Amino acid metabolism > Urea cyclecerebellum, brainstem, spinalcord, liver, heart, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta
MRPL14mitochondrial ­ribosomal ­protein ­L14L14mt, L32mt, MRP-L14, MRP-L32, MRPL32, RMPL32, RPML32, Q6P1L8, gene:6492815literature, APEX_matrix, targetP signal, Rickettsial homolog, mito protein domain, coexpression+, MS/MSMatrixMitochondrial central dogma > Translation > Mitochondrial ribosomeall 14
MTRES1mitochondrial ­transcription ­rescue ­factor ­1C6orf203, HSPC230, PRED31, Q9P0P8, gene:5125015GFP, induction, coexpression++, MS/MS+MatrixMitochondrial central dogma > mtRNA metabolism > Transcription;
Mitochondrial central dogma > Translation
all 14
RDH13retinol ­dehydrogenase ­13SDR7C3, Q8NBN7, gene:11272415yeast mito homolog+, Rickettsial homolog, mito protein domain, induction, MS/MSMIMMetabolism > Vitamin metabolism > Vitamin A metabolismcerebrum, cerebellum, brainstem, spinalcord, adipose, largeintestine, stomach, placenta, testis
TRMT2BtRNA ­methyltransferase ­2 ­homolog ­BCXorf34, dJ341D10.3, Q96GJ1, gene:7997915GFP, targetP signal, Rickettsial homolog, mito protein domain+, induction, coexpressionMatrixMitochondrial central dogma > mtRNA metabolism > mt-tRNA modifications;
Mitochondrial central dogma > mtRNA metabolism > mt-rRNA modifications
none
MRPS24mitochondrial ­ribosomal ­protein ­S24HSPC335, MRP-S24, S24mt, bMRP-47, bMRP47, Q96EL2, gene:6495115literature, APEX_matrix, induction, coexpression++, MS/MSMatrixMitochondrial central dogma > Translation > Mitochondrial ribosomecerebrum, cerebellum, brainstem, spinalcord, kidney, liver, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
COA3cytochrome ­c ­oxidase ­assembly ­factor ­3CCDC56, COX25, HSPC009, MITRAC12, Q9Y2R0, gene:2895815APEX_IMS, APEX_matrix, mito protein domain+, coexpression, MS/MS++MIMMitochondrial central dogma > Translation > Translation factors;
OXPHOS > Complex IV > CIV assembly factors;
OXPHOS > OXPHOS assembly factors
all 14
GSRglutathione-disulfide ­reductaseGR, GSRD, HEL-75, HEL-S-122m, P00390, gene:293615literature, APEX_matrix, targetP signal, Rickettsial homolog, mito protein domain, MS/MS++MatrixMetabolism > Detoxification > ROS and glutathione metabolismspinalcord, kidney, liver, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
ENDOGendonuclease ­GQ14249, gene:202115literature, APEX_IMS, mito protein domain, coexpression++, MS/MSIMSMitochondrial central dogma > mtDNA maintenance > mtDNA stability and decay;
Mitochondrial central dogma > mtRNA metabolism > mtRNA stability and decay;
Mitochondrial dynamics and surveillance > Apoptosis
all 14
BCAT2branched ­chain ­amino ­acid ­transaminase ­2BCAM, BCATM, BCT2, HVLI, PP18, O15382, gene:58715literature, APEX_matrix, targetP signal, Rickettsial homolog, mito protein domain, coexpression, MS/MS++MatrixMetabolism > Amino acid metabolism > BCAA metabolismcerebrum, cerebellum, brainstem, kidney, heart, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
TEFMtranscription ­elongation ­factor, ­mitochondrialC17orf42, Q96QE5, gene:7973615APEX_matrix, targetP signal, induction, coexpression++, MS/MSMatrixMitochondrial central dogma > mtRNA metabolism > Transcriptionliver, heart, skeletalmuscle, adipose, largeintestine, stomach, placenta, testis
ACSF2acyl-CoA ­synthetase ­family ­member ­2ACSMW, AVYV493, Q96CM8, gene:8022115APEX_matrix, targetP signal+, Rickettsial homolog, mito protein domain, MS/MS++MatrixMetabolism > Lipid metabolism > Fatty acid oxidationcerebrum, cerebellum, brainstem, spinalcord, kidney, liver, heart, adipose, smallintestine, largeintestine, stomach, placenta, testis
TRMUtRNA ­mitochondrial ­2-thiouridylaseLCAL3, MTO2, MTU1, TRMT, TRMT1, O75648, gene:5568715literature, APEX_matrix, yeast mito homolog++, Rickettsial homolog, mito protein domain, coexpressionMatrixMitochondrial central dogma > mtRNA metabolism > mt-tRNA modificationsadipose, largeintestine, placenta
MRPS22mitochondrial ­ribosomal ­protein ­S22C3orf5, COXPD5, GIBT, GK002, MRP-S22, ODG7, RPMS22, P82650, gene:5694515literature, APEX_matrix, mito protein domain+, induction, coexpression, MS/MSMatrixMitochondrial central dogma > Translation > Mitochondrial ribosomeall 14
LAP3leucine ­aminopeptidase ­3HEL-S-106, LAP, LAPEP, PEPS, P28838, gene:5105615APEX_matrix, targetP signal+, Rickettsial homolog, coexpression, MS/MS++MatrixProtein import, sorting and homeostasis > Protein homeostasis > Proteasesall 14
GLUD1glutamate ­dehydrogenase ­1GDH, GDH1, GLUD, P00367, gene:274615literature, APEX_matrix, Rickettsial homolog, mito protein domain, coexpression, MS/MS++MatrixMetabolism > Amino acid metabolism > Glutamate metabolism;
Metabolism > Amino acid metabolism > GABA metabolism
all 14
HMGCL3-hydroxy-3-methylglutaryl-CoA ­lyaseHL, P35914, gene:315515literature, APEX_matrix, targetP signal+, mito protein domain, coexpression, MS/MS++MatrixMetabolism > Carbohydrate metabolism > Ketone metabolism;
Metabolism > Amino acid metabolism > BCAA metabolism
all 14
FHITfragile ­histidine ­triad ­diadenosine ­triphosphataseAP3Aase, FRA3B, P49789, gene:227215yeast mito homolog, Rickettsial homolog, mito protein domain, MS/MS++MatrixMetabolism > Nucleotide metabolism > Nucleotide synthesis and processingcerebrum, cerebellum, brainstem, spinalcord, kidney, liver, adipose, smallintestine, largeintestine, stomach, placenta, testis
MRPS33mitochondrial ­ribosomal ­protein ­S33CGI-139, MRP-S33, PTD003, S33mt, Q9Y291, gene:5165015literature, targetP signal, mito protein domain+, MS/MS++MatrixMitochondrial central dogma > Translation > Mitochondrial ribosomecerebrum, cerebellum, brainstem, spinalcord, kidney, liver, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
NUBPLnucleotide ­binding ­protein ­likeC14orf127, IND1, MC1DN21, huInd1, Q8TB37, gene:8022414literature, targetP signal, Rickettsial homolog, mito protein domain, coexpression+, MS/MS+MatrixOXPHOS > Complex I > CI assembly factors;
Metabolism > Metals and cofactors > Fe-S cluster biosynthesis;
Metabolism > Metals and cofactors > Fe-S-containing proteins;
OXPHOS > OXPHOS assembly factors
spinalcord, adipose, largeintestine, stomach, placenta, testis
LYRM2LYR ­motif ­containing ­2DJ122O8.2, Q9NU23, gene:5722614APEX_matrix, GFP, targetP signal+, mito protein domain+, coexpression, MS/MSMatrixOXPHOS > Complex I > CI assembly factors;
OXPHOS > OXPHOS assembly factors
heart, adipose, largeintestine, placenta
MPV17LMPV17 ­mitochondrial ­inner ­membrane ­protein ­likeM-LPH, MLPH1, MLPH2, MPV17L1, Q2QL34, gene:25502714targetP signal, yeast mito homolog+, mito protein domain, MS/MS++MIMSmall molecule transportcerebrum, cerebellum, brainstem, spinalcord, kidney, liver
GADD45GIP1GADD45G ­interacting ­protein ­1CKBBP2, CKbetaBP2, CRIF1, MRP-L59, PLINP, PLINP-1, PRG6, Plinp1, Q8TAE8, gene:9048014APEX_matrix, GFP, targetP signal, coexpression+, MS/MS++MatrixMitochondrial central dogma > Translation > Mitochondrial ribosomeall 14
TFAMtranscription ­factor ­A, ­mitochondrialMTDPS15, MTTF1, MTTFA, TCF6, TCF6L1, TCF6L2, TCF6L3, Q00059, gene:701914literature, APEX_matrix, targetP signal, mito protein domain, induction, MS/MS++MatrixMitochondrial central dogma > mtDNA maintenance > mtDNA replication;
Mitochondrial central dogma > mtDNA maintenance > mtDNA nucleoid;
Mitochondrial central dogma > mtRNA metabolism > Transcription
all 14
ALDH18A1aldehyde ­dehydrogenase ­18 ­family ­member ­A1ADCL3, ARCL3A, GSAS, P5CS, PYCS, SPG9, SPG9A, SPG9B, P54886, gene:583214literature, Rickettsial homolog, mito protein domain, coexpression, MS/MS++MatrixMetabolism > Amino acid metabolism > Proline metabolismcerebrum, cerebellum, brainstem, spinalcord, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
OXSM3-oxoacyl-ACP ­synthase, ­mitochondrialCEM1, FASN2D, KASI, KS, Q9NWU1, gene:5499514literature, APEX_matrix, targetP signal, Rickettsial homolog, mito protein domain, MS/MS++MatrixMetabolism > Lipid metabolism > Type II fatty acid synthesisliver, heart, adipose, smallintestine, largeintestine, placenta, testis
KYAT3kynurenine ­aminotransferase ­3CCBL2, KAT3, KATIII, Q6YP21, gene:5626714APEX_matrix, targetP signal, Rickettsial homolog, mito protein domain, MS/MS++MatrixMetabolism > Amino acid metabolism > Kynurenine metabolism;
Metabolism > Metals and cofactors > NAD biosynthesis and metabolism
spinalcord, kidney, liver, heart, adipose, smallintestine, largeintestine, stomach, placenta, testis
TARS2threonyl-tRNA ­synthetase ­2, ­mitochondrialCOXPD21, TARSL1, thrRS, Q9BW92, gene:8022214APEX_matrix, targetP signal, Rickettsial homolog, mito protein domain, MS/MS++MatrixMitochondrial central dogma > Translation > mt-tRNA synthetasescerebrum, cerebellum, brainstem, spinalcord, kidney, liver, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
TBRG4transforming ­growth ­factor ­beta ­regulator ­4CPR2, FASTKD4, Q969Z0, gene:923814literature, APEX_matrix, targetP signal+, coexpression, MS/MS++MatrixMitochondrial central dogma > mtRNA metabolism > Polycistronic mtRNA processing;
Mitochondrial central dogma > mtRNA metabolism > mtRNA stability and decay
all 14
TMEM65transmembrane ­protein ­65Q6PI78, gene:15737814literature, APEX_matrix, targetP signal+, coexpression, MS/MS++MIMnonecerebrum, cerebellum, brainstem, spinalcord, kidney, heart, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
ROMO1reactive ­oxygen ­species ­modulator ­1C20orf52, MTGM, MTGMP, bA353C18.2, P60602, gene:14082314literature, APEX_IMS, yeast mito homolog++, mito protein domain+, MS/MSMIMProtein import, sorting and homeostasis > Protein import and sorting > TIM23 presequence pathway;
Small molecule transport
cerebrum, cerebellum, brainstem, spinalcord, liver, skeletalmuscle, adipose, smallintestine, stomach, placenta, testis
PRDX5peroxiredoxin ­5ACR1, AOEB166, B166, HEL-S-55, PLP, PMP20, PRDX6, PRXV, SBBI10, prx-V, P30044, gene:2582414literature, APEX_matrix, targetP signal, mito protein domain, induction, MS/MS++MatrixMetabolism > Detoxification > ROS and glutathione metabolismall 14
DNAJC15DnaJ ­heat ­shock ­protein ­family ­(Hsp40) ­member ­C15DNAJD1, HSD18, MCJ, Q9Y5T4, gene:2910314GFP, mito protein domain, induction, coexpression, MS/MS+MIMProtein import, sorting and homeostasis > Protein import and sorting > Import motorcerebrum, spinalcord, liver, adipose, smallintestine, largeintestine, placenta, testis
MAIP1matrix ­AAA ­peptidase ­interacting ­protein ­1C2orf47, Q8WWC4, gene:7956814GFP, targetP signal, induction, coexpression, MS/MS+MatrixProtein import, sorting and homeostasis > Protein homeostasis > Chaperonesall 14
HIGD1AHIG1 ­hypoxia ­inducible ­domain ­family ­member ­1AHIG1, RCF1a, Q9Y241, gene:2599414literature, APEX_IMS, mito protein domain+, induction, MS/MS++MIMOXPHOS > Complex IV > CIV assembly factors;
OXPHOS > OXPHOS assembly factors;
OXPHOS > Respirasome assembly
all 14
C16orf91chromosome ­16 ­open ­reading ­frame ­91CCSMST1, URLC5, gs103, Q4G0I0, gene:28395114APEX_IMS, targetP signal+, induction, coexpression, MS/MSIMSnonebrainstem, spinalcord, adipose, largeintestine, placenta, testis
RHOT2ras ­homolog ­family ­member ­T2ARHT2, C16orf39, MIRO-2, MIRO2, RASL, Q8IXI1, gene:8994114literature, mito protein domain+, induction, coexpressionMOMSignaling > Calcium homeostasis > EF hand proteins;
Mitochondrial dynamics and surveillance > Trafficking
cerebrum, cerebellum, brainstem, spinalcord, kidney, liver, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
SLC25A44solute ­carrier ­family ­25 ­member ­44Q96H78, gene:967314yeast mito homolog+, mito protein domain+, induction, MS/MSMIMMetabolism > Amino acid metabolism > BCAA metabolism;
Small molecule transport > SLC25A family
placenta
HAO2hydroxyacid ­oxidase ­2GIG16, HAOX2, Q9NYQ3, gene:5117914yeast mito homolog+, Rickettsial homolog, mito protein domain, coexpression, MS/MSunknownMetabolism > Lipid metabolismkidney, largeintestine, testis
OXA1LOXA1L ­mitochondrial ­inner ­membrane ­proteinOXA1, Q15070, gene:501814literature, APEX_matrix, targetP signal, yeast mito homolog+, Rickettsial homolog, mito protein domain, coexpressionMIMProtein import, sorting and homeostasis > Protein import and sorting > OXA;
Mitochondrial central dogma > Translation
cerebrum, brainstem, spinalcord, kidney, heart, adipose, smallintestine, largeintestine, placenta, testis
SLC25A46solute ­carrier ­family ­25 ­member ­46HMSN6B, Q96AG3, gene:9113714yeast mito homolog++, mito protein domain+, inductionMOMSmall molecule transport > SLC25A family;
Mitochondrial dynamics and surveillance > Fission;
Mitochondrial dynamics and surveillance > Intramitochondrial membrane interactions
cerebrum, spinalcord, adipose, placenta
RDH14retinol ­dehydrogenase ­14PAN2, SDR7C4, Q9HBH5, gene:5766514yeast mito homolog+, Rickettsial homolog, mito protein domain, coexpression, MS/MSMembraneMetabolism > Vitamin metabolism > Vitamin A metabolismcerebrum, cerebellum, brainstem, spinalcord, smallintestine, largeintestine, stomach, placenta, testis
ATP5MPLATP ­synthase ­membrane ­subunit ­6.8PL6.8PL, C14orf2, MLQ, MP68, PLPM, P56378, gene:955614induction, coexpression++, MS/MS+MIMOXPHOS > Complex V > CV subunits;
OXPHOS > OXPHOS subunits
cerebrum, cerebellum, brainstem, spinalcord, kidney, liver, heart, adipose, smallintestine, largeintestine, stomach, placenta
CA5Bcarbonic ­anhydrase ­5BCA-VB, CAVB, Q9Y2D0, gene:1123814literature, targetP signal, mito protein domain, coexpression, MS/MS++MatrixMetabolism > Amino acid metabolism > Urea cyclecerebellum, spinalcord, kidney, adipose, stomach, placenta, testis
COASYCoenzyme ­A ­synthaseDPCK, NBIA6, NBP, PCH12, PPAT, UKR1, pOV-2, Q13057, gene:8034714APEX_matrix, targetP signal, mito protein domain, coexpression, MS/MS++MatrixMetabolism > Metals and cofactors > Coenzyme A metabolismcerebrum, cerebellum, brainstem, spinalcord, kidney, liver, adipose, smallintestine, largeintestine, stomach, placenta, testis
CISD3CDGSH ­iron ­sulfur ­domain ­3MiNT, Miner2, P0C7P0, gene:28410614APEX_matrix, targetP signal, mito protein domain, coexpression, MS/MS++MatrixMetabolism > Metals and cofactors > Fe-S cluster biosynthesis;
Metabolism > Metals and cofactors > Fe-S-containing proteins
all 14
GSTZ1glutathione ­S-transferase ­zeta ­1GSTZ1-1, MAAI, MAAID, MAI, O43708, gene:295414APEX_matrix, mito protein domain, coexpression, MS/MS++MatrixMetabolism > Detoxification > Xenobiotic metabolismall 14
COX6B2cytochrome ­c ­oxidase ­subunit ­6B2COXVIB2, CT59, Q6YFQ2, gene:12596514literature, yeast mito homolog+, mito protein domain+, MS/MS+MIMOXPHOS > Complex IV > CIV subunits;
OXPHOS > OXPHOS subunits
testis
GLOD4glyoxalase ­domain ­containing ­4C17orf25, CGI-150, HC71, Q9HC38, gene:5103114APEX_matrix, targetP signal+, coexpression, MS/MS++Matrixnonecerebrum, cerebellum, brainstem, spinalcord, kidney, liver, heart, adipose, smallintestine, largeintestine, stomach, placenta, testis
ACAA1acetyl-CoA ­acyltransferase ­1ACAA, PTHIO, THIO, P09110, gene:3014yeast mito homolog++, Rickettsial homolog, mito protein domain, coexpressionunknownMetabolism > Lipid metabolismkidney, liver, adipose, smallintestine, largeintestine, stomach, placenta, testis
CROTcarnitine ­O-octanoyltransferaseCOT, Q9UKG9, gene:5467714yeast mito homolog+, mito protein domain, induction, coexpression, MS/MSunknownMetabolism > Lipid metabolism > Fatty acid oxidationkidney, liver
PDFpeptide ­deformylase, ­mitochondrialQ9HBH1, gene:6414614APEX_matrix, Rickettsial homolog, mito protein domain+, coexpression, MS/MSMatrixMitochondrial central dogma > Translation > fMet processing;
Protein import, sorting and homeostasis > Protein homeostasis
cerebrum, heart, skeletalmuscle, adipose, largeintestine, placenta, testis
COX19cytochrome ­c ­oxidase ­assembly ­factor ­COX19Q49B96, gene:9063914yeast mito homolog, mito protein domain, induction, coexpression+, MS/MSIMSOXPHOS > Complex IV > CIV assembly factors;
Metabolism > Metals and cofactors > Copper metabolism;
OXPHOS > OXPHOS assembly factors
liver, placenta
AMTaminomethyltransferaseGCE, GCST, GCVT, NKH, P48728, gene:27514targetP signal+, mito protein domain, MS/MS++MatrixMetabolism > Amino acid metabolism > Glycine metabolism;
Metabolism > Amino acid metabolism > Glycine cleavage system;
Metabolism > Vitamin metabolism > Folate and 1-C metabolism
cerebrum, brainstem, spinalcord, kidney, liver, largeintestine, stomach, placenta
PDPRpyruvate ­dehydrogenase ­phosphatase ­regulatory ­subunitPDP3, Q8NCN5, gene:5506614APEX_matrix, targetP signal+, mito protein domain, MS/MS++MatrixMetabolism > Carbohydrate metabolism > Pyruvate metabolismcerebrum, cerebellum, brainstem, spinalcord, heart, skeletalmuscle, adipose, largeintestine, stomach, placenta, testis
NLNneurolysinAGTBP, EP24.16, MEP, MOP, Q9BYT8, gene:5748614literature, APEX_matrix, targetP signal+, mito protein domain, MS/MS++MatrixProtein import, sorting and homeostasis > Protein homeostasis > Proteasescerebrum, cerebellum, brainstem, spinalcord, kidney, liver, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
BOLA1bolA ­family ­member ­1CGI-143, Q9Y3E2, gene:5102713GFP, Rickettsial homolog, mito protein domain, coexpression, MS/MS++MatrixMetabolism > Metals and cofactors > Fe-S cluster biosynthesis;
Metabolism > Metals and cofactors > Fe-S-containing proteins
cerebrum, brainstem, spinalcord, kidney, liver, heart, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
ALAS25'-aminolevulinate ­synthase ­2ALAS-E, ALASE, ANH1, ASB, SIDBA1, XLDPP, XLEPP, XLSA, P22557, gene:21213literature, yeast mito homolog+, Rickettsial homolog, mito protein domain+, coexpressionMatrixMetabolism > Metals and cofactors > Heme synthesis and processingnone
GRPEL2GrpE ­like ­2, ­mitochondrialMt-GrpE#2, Q8TAA5, gene:13426613literature, APEX_matrix, targetP signal+, Rickettsial homolog, mito protein domain+MatrixProtein import, sorting and homeostasis > Protein import and sorting > Import motor;
Metabolism > Metals and cofactors > Fe-S cluster biosynthesis
placenta
ALDH7A1aldehyde ­dehydrogenase ­7 ­family ­member ­A1ATQ1, EPD, PDE, P49419, gene:50113literature, APEX_matrix, targetP signal, yeast mito homolog, mito protein domain, MS/MS++MatrixMetabolism > Amino acid metabolism > Lysine metabolism;
Metabolism > Amino acid metabolism > Glycine metabolism;
Metabolism > Vitamin metabolism > Choline and betaine metabolism;
Metabolism > Vitamin metabolism > Folate and 1-C metabolism;
Metabolism > Detoxification > ROS and glutathione metabolism
cerebrum, cerebellum, brainstem, spinalcord, kidney, liver, adipose, smallintestine, largeintestine, stomach, placenta, testis
HDHD5haloacid ­dehalogenase ­like ­hydrolase ­domain ­containing ­5CECR5, Q9BXW7, gene:2744013APEX_matrix, targetP signal, yeast mito homolog, mito protein domain, MS/MS++Matrixnonecerebellum, kidney, liver, skeletalmuscle, adipose, largeintestine, stomach, placenta, testis
GFERgrowth ­factor, ­augmenter ­of ­liver ­regenerationALR, ERV1, HERV1, HPO, HPO1, HPO2, HSS, MMCHD, MPMCD, P55789, gene:267113literature, GFP, yeast mito homolog++, mito protein domain, coexpression, MS/MSIMSProtein import, sorting and homeostasis > Protein import and sorting > MIA40cerebrum, cerebellum, brainstem, spinalcord, kidney, liver, adipose, smallintestine, largeintestine, stomach, placenta, testis
IMMP2Linner ­mitochondrial ­membrane ­peptidase ­subunit ­2IMMP2L-IT1, IMP2, IMP2-LIKE, Q96T52, gene:8394313literature, yeast mito homolog++, mito protein domain, coexpression, MS/MSMIMProtein import, sorting and homeostasis > Protein import and sorting > Preprotein cleavage;
Protein import, sorting and homeostasis > Protein homeostasis > Proteases
cerebellum, brainstem, liver, skeletalmuscle, adipose, largeintestine, testis
COX4I2cytochrome ­c ­oxidase ­subunit ­4I2COX4, COX4-2, COX4B, COX4L2, COXIV-2, dJ857M17.2, Q96KJ9, gene:8470113literature, targetP signal, yeast mito homolog++, mito protein domain+MIMOXPHOS > Complex IV > CIV subunits;
OXPHOS > OXPHOS subunits
cerebrum, spinalcord, heart, adipose, largeintestine, stomach, placenta
MRPL39mitochondrial ­ribosomal ­protein ­L39C21orf92, L39mt, L5mt, MRP-L5, MRPL5, MSTP003, PRED22, PRED66, RPML5, Q9NYK5, gene:5414813literature, APEX_matrix, targetP signal, Rickettsial homolog, induction, coexpression, MS/MSMatrixMitochondrial central dogma > Translation > Mitochondrial ribosomecerebrum, cerebellum, brainstem, kidney, liver, heart, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
AADATaminoadipate ­aminotransferaseKAT2, KATII, KYAT2, Q8N5Z0, gene:5116613targetP signal, Rickettsial homolog, mito protein domain, coexpression, MS/MS++MatrixMetabolism > Amino acid metabolism > Lysine metabolismkidney, liver
FUNDC2FUN14 ­domain ­containing ­2DC44, HCBP6, HCC3, PD03104, Q9BWH2, gene:6599113mito protein domain+, MS/MS++MOMMitochondrial dynamics and surveillance > Organelle contact sites;
Signaling
cerebrum, cerebellum, brainstem, spinalcord, kidney, heart, skeletalmuscle, adipose, largeintestine, stomach, placenta, testis
LYPLA1lysophospholipase ­1APT-1, APT1, LPL-I, LPL1, hAPT1, O75608, gene:1043413Rickettsial homolog, mito protein domain, coexpression, MS/MS++unknownMetabolism > Lipid metabolism;
Signaling
all 14
PDHA2pyruvate ­dehydrogenase ­E1 ­subunit ­alpha ­2PDHAL, P29803, gene:516113targetP signal+, yeast mito homolog+, Rickettsial homolog, mito protein domain+MatrixMetabolism > Carbohydrate metabolism > Pyruvate metabolismnone
MTRF1Lmitochondrial ­translational ­release ­factor ­1 ­likeHMRF1L, MRF1L, mtRF1a, Q9UGC7, gene:5451613literature, targetP signal+, yeast mito homolog+, Rickettsial homolog, mito protein domainMatrixMitochondrial central dogma > Translation > Translation factorstestis
GTPBP3GTP ­binding ­protein ­3, ­mitochondrialCOXPD23, GTPBG3, MSS1, MTGP1, THDF1, Q969Y2, gene:8470513literature, APEX_matrix, targetP signal+, yeast mito homolog+, Rickettsial homolog, mito protein domainMatrixMitochondrial central dogma > mtRNA metabolism > mt-tRNA modifications;
Metabolism > Vitamin metabolism > Folate and 1-C metabolism
largeintestine
MTHFD2Lmethylenetetrahydrofolate ­dehydrogenase ­(NADP+ ­dependent) ­2 ­likeQ9H903, gene:44102413literature, targetP signal+, yeast mito homolog+, Rickettsial homolog, mito protein domainMatrixMetabolism > Vitamin metabolism > Folate and 1-C metabolismspinalcord, placenta
YME1L1YME1 ­like ­1 ­ATPaseFTSH, MEG4, OPA11, PAMP, YME1L, Q96TA2, gene:1073013literature, APEX_IMS, Rickettsial homolog, mito protein domain+, MS/MS+MIMProtein import, sorting and homeostasis > Protein homeostasis > Proteasescerebrum, cerebellum, brainstem, spinalcord, kidney, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
ACOT2acyl-CoA ­thioesterase ­2CTE-IA, CTE1A, MTE1, PTE2, PTE2A, ZAP128, P49753, gene:1096513literature, mito protein domain, induction, coexpression, MS/MS++MatrixMetabolism > Lipid metabolismall 14
COA8cytochrome ­c ­oxidase ­assembly ­factor ­8APOP, APOP1, APOPT1, C14orf153, Q96IL0, gene:8433413literature, targetP signal, mito protein domain+, coexpression+MIMOXPHOS > Complex IV > CIV assembly factors;
OXPHOS > OXPHOS assembly factors
largeintestine, placenta
MMABmetabolism ­of ­cobalamin ­associated ­BATR, CFAP23, cblB, cob, Q96EY8, gene:32662513APEX_matrix, GFP, targetP signal, coexpression, MS/MS++MatrixMetabolism > Vitamin metabolism > Vitamin B12 metabolismcerebrum, cerebellum, brainstem, spinalcord, kidney, liver, heart, skeletalmuscle, adipose, largeintestine, stomach, placenta, testis
PPOXprotoporphyrinogen ­oxidasePPO, V290M, VP, P50336, gene:549813literature, APEX_matrix, yeast mito homolog++, mito protein domain, coexpression, MS/MSMIMMetabolism > Metals and cofactors > Heme synthesis and processingcerebrum, spinalcord, liver, adipose, smallintestine, largeintestine, stomach, placenta, testis
ABCD3ATP ­binding ­cassette ­subfamily ­D ­member ­3ABC43, CBAS5, PMP70, PXMP1, ZWS2, P28288, gene:582513GFP, targetP signal, Rickettsial homolog, mito protein domain, induction, coexpressionMembraneSmall molecule transport > ABC transporterskidney, liver, adipose, placenta, testis
SIRT3sirtuin ­3SIR2L3, Q9NTG7, gene:2341013literature, APEX_matrix, mito protein domain, induction, coexpression, MS/MS+MatrixMetabolism > Metals and cofactors > NAD biosynthesis and metabolism;
Signaling
cerebrum, cerebellum, brainstem, spinalcord, kidney, liver, heart, adipose, smallintestine, largeintestine, stomach, placenta, testis
LDHDlactate ­dehydrogenase ­DDLACD, DLD, Q86WU2, gene:19725713literature, yeast mito homolog+, mito protein domain, MS/MS++MIMMetabolism > Carbohydrate metabolismkidney, liver, heart, adipose, stomach, placenta, testis
MRPL45mitochondrial ­ribosomal ­protein ­L45L45mt, MRP-L45, B4DEF8, gene:8431113literature, APEX_matrix, mito protein domain, induction, coexpression, MS/MS+MatrixMitochondrial central dogma > Translation > Mitochondrial ribosomecerebrum, brainstem, spinalcord, adipose, smallintestine, largeintestine, stomach, placenta, testis
C12orf65chromosome ­12 ­open ­reading ­frame ­65COXPD7, SPG55, Q9H3J6, gene:9157413literature, yeast mito homolog++, Rickettsial homolog, mito protein domain, MS/MSMatrixMitochondrial central dogma > Translation > Translation factorscerebrum, brainstem, spinalcord, adipose, largeintestine, stomach, placenta
IMMP1Linner ­mitochondrial ­membrane ­peptidase ­subunit ­1IMMP1, IMP1, IMP1-LIKE, Q96LU5, gene:19629413literature, yeast mito homolog++, Rickettsial homolog, mito protein domain, MS/MSMIMProtein import, sorting and homeostasis > Protein import and sorting > Preprotein cleavage;
Protein import, sorting and homeostasis > Protein homeostasis > Proteases
testis
POLGDNA ­polymerase ­gamma, ­catalytic ­subunitMDP1, MIRAS, MTDPS4A, MTDPS4B, PEO, POLG1, POLGA, SANDO, SCAE, P54098, gene:542813literature, APEX_matrix, targetP signal, yeast mito homolog++, mito protein domain, MS/MSMatrixMitochondrial central dogma > mtDNA maintenance > mtDNA replication;
Mitochondrial central dogma > mtDNA maintenance > mtDNA nucleoid;
Mitochondrial central dogma > mtDNA maintenance > mtDNA repair
cerebrum, cerebellum, spinalcord, liver, adipose, largeintestine, placenta, testis
ATPAF1ATP ­synthase ­mitochondrial ­F1 ­complex ­assembly ­factor ­1ATP11, ATP11p, Q5TC12, gene:6475613literature, APEX_matrix, yeast mito homolog++, mito protein domain+, MS/MSMatrixOXPHOS > Complex V > CV assembly factors;
OXPHOS > OXPHOS assembly factors
cerebrum, brainstem, spinalcord, adipose, smallintestine, largeintestine, placenta, testis
BLOC1S1biogenesis ­of ­lysosomal ­organelles ­complex ­1 ­subunit ­1BLOS1, BORCS1, GCN5L1, MICoA, RT14, P78537, gene:264713mito protein domain, coexpression++, MS/MSunknownSignalingplacenta, testis
ALDH1L2aldehyde ­dehydrogenase ­1 ­family ­member ­L2mtFDH, Q3SY69, gene:16042813literature, APEX_matrix, targetP signal, Rickettsial homolog, mito protein domain, coexpression, MS/MSMatrixMetabolism > Vitamin metabolism > Folate and 1-C metabolismbrainstem, spinalcord, liver, skeletalmuscle, smallintestine, largeintestine, stomach, placenta
COX18cytochrome ­c ­oxidase ­assembly ­factor ­COX18COX18HS, Q8N8Q8, gene:28552113literature, targetP signal, yeast mito homolog+, Rickettsial homolog, mito protein domain, coexpressionMIMOXPHOS > Complex IV > CIV assembly factors;
OXPHOS > OXPHOS assembly factors
none
CPOXcoproporphyrinogen ­oxidaseCOX, CPO, CPX, HARPO, HCP, P36551, gene:137113literature, APEX_IMS, APEX_matrix, Rickettsial homolog, mito protein domain, coexpression, MS/MS++MIMMetabolism > Metals and cofactors > Heme synthesis and processingcerebellum, brainstem, spinalcord, kidney, liver, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
NNTnicotinamide ­nucleotide ­transhydrogenaseGCCD4, Q13423, gene:2353013literature, APEX_matrix, Rickettsial homolog, mito protein domain, induction, coexpression, MS/MS+MIMMetabolism > Metals and cofactors > NAD biosynthesis and metabolismcerebrum, spinalcord, skeletalmuscle, adipose, placenta
NDUFAF8NADH:ubiquinone ­oxidoreductase ­complex ­assembly ­factor ­8C17orf89, MC1DN34, A1L188, gene:28418413targetP signal, induction, coexpression+, MS/MSIMSOXPHOS > Complex I > CI assembly factors;
OXPHOS > OXPHOS assembly factors
adipose, stomach
ATP23ATP23 ­metallopeptidase ­and ­ATP ­synthase ­assembly ­factor ­homologKUB3, XRCC6BP1, Q9Y6H3, gene:9141913yeast mito homolog++, mito protein domain+, coexpressionIMSProtein import, sorting and homeostasis > Protein homeostasis > Proteasescerebellum
DCAKDdephospho-CoA ­kinase ­domain ­containingQ8WVC6, gene:7987713GFP, yeast mito homolog, Rickettsial homolog, mito protein domain, coexpression, MS/MSMembranenoneall 14
ATP5MFATP ­synthase ­membrane ­subunit ­fATP5J2, ATP5JL, P56134, gene:955113literature, coexpression++, MS/MS++MIMOXPHOS > Complex V > CV subunits;
OXPHOS > OXPHOS subunits
all 14
SFXN3sideroflexin ­3BA108L7.2, SFX3, SLC56A3, Q9BWM7, gene:8185513mito protein domain+, MS/MS++MIMMetabolism > Amino acid metabolism > Serine metabolism;
Metabolism > Vitamin metabolism > Folate and 1-C metabolism;
Small molecule transport > Sideroflexins
cerebrum, cerebellum, brainstem, spinalcord, kidney, liver, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
AASSaminoadipate-semialdehyde ­synthaseLKR/SDH, LKRSDH, LORSDH, Q9UDR5, gene:1015713APEX_matrix, targetP signal, mito protein domain, coexpression, MS/MS++MatrixMetabolism > Amino acid metabolism > Lysine metabolismcerebrum, cerebellum, brainstem, spinalcord, kidney, liver, adipose, largeintestine, stomach, placenta, testis
DIABLOdiablo ­IAP-binding ­mitochondrial ­proteinDFNA64, SMAC, Q9NR28, gene:5661613literature, APEX_IMS, targetP signal+, mito protein domain+, MS/MSIMSMitochondrial dynamics and surveillance > Apoptosiscerebrum, cerebellum, brainstem, spinalcord, liver, heart, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
COX8Ccytochrome ­c ­oxidase ­subunit ­8CCOX8-3, Q7Z4L0, gene:34194713literature, targetP signal+, mito protein domain+, MS/MSMIMOXPHOS > Complex IV > CIV subunits;
OXPHOS > OXPHOS subunits
testis
PXMP2peroxisomal ­membrane ­protein ­2MPV17L3, PMP22, Q9NR77, gene:582713GFP, targetP signal, yeast mito homolog+, mito protein domain, inductionMIMnonecerebrum, cerebellum, brainstem, spinalcord, kidney, liver, heart, skeletalmuscle, adipose, smallintestine, largeintestine, placenta, testis
AGXT2alanine--glyoxylate ­aminotransferase ­2AGT2, BAIBA, DAIBAT, Q9BYV1, gene:6490213literature, yeast mito homolog++, mito protein domain, MS/MS+MatrixMetabolism > Amino acid metabolism > Glyoxylate metabolismkidney, liver
GLS2glutaminase ­2GA, GLS, LGA, hLGA, Q9UI32, gene:2716513targetP signal, mito protein domain+, MS/MS++MatrixMetabolism > Amino acid metabolism > Glutamate metabolismcerebrum, cerebellum, brainstem, spinalcord, liver, skeletalmuscle, smallintestine, largeintestine
APOOapolipoprotein ­OFAM121B, MIC26, MICOS26, Mic23, My025, Q9BUR5, gene:7913513literature, APEX_IMS, mito protein domain+, MS/MS++MIMMitochondrial dynamics and surveillance > Cristae formation > MICOS complexall 14
ACAD11acyl-CoA ­dehydrogenase ­family ­member ­11ACAD-11, Q709F0, gene:8412913mito protein domain+, induction, coexpressionMIMMetabolism > Lipid metabolism > Fatty acid oxidationspinalcord, kidney, liver, adipose
C5orf63chromosome ­5 ­open ­reading ­frame ­63YDR286C, A6NC05, gene:40120713targetP signal+, coexpression+, MS/MSMatrixnonecerebrum, cerebellum, spinalcord, liver, heart, skeletalmuscle, adipose, placenta, testis
HOGA14-hydroxy-2-oxoglutarate ­aldolase ­1C10orf65, DHDPS2, DHDPSL, HP3, NPL2, Q86XE5, gene:11281713GFP, targetP signal, Rickettsial homolog, mito protein domain, coexpression, MS/MSMatrixMetabolism > Amino acid metabolism > Glyoxylate metabolismkidney, liver, adipose, largeintestine, placenta, testis
TTC19tetratricopeptide ­repeat ­domain ­192010204O13Rik, MC3DN2, Q6DKK2, gene:5490213literature, APEX_IMS, targetP signal+, mito protein domain, induction, MS/MS+MIMOXPHOS > Complex III > CIII assembly factors;
OXPHOS > OXPHOS assembly factors
cerebrum, cerebellum, spinalcord, adipose, largeintestine, stomach, testis
NGRNneugrin, ­neurite ­outgrowth ­associatedDSC92, Q9NPE2, gene:5133512APEX_matrix, yeast mito homolog++, mito protein domain+, coexpressionMatrixMitochondrial central dogma > mtRNA metabolism > mt-rRNA modifications;
Mitochondrial central dogma > Translation > Mitochondrial ribosome assembly
none
TCAIMT ­cell ­activation ­inhibitor, ­mitochondrialC3orf23, TOAG-1, TOAG1, Q8N3R3, gene:28534312literature, APEX_matrix, targetP signal, mito protein domain+, coexpression++Matrixnonenone
GCSHglycine ­cleavage ­system ­protein ­HGCE, NKH, P23434, gene:265312literature, APEX_matrix, targetP signal, yeast mito homolog, mito protein domain+, MS/MSMatrixMetabolism > Lipid metabolism > Lipoate insertion;
Metabolism > Amino acid metabolism > Glycine metabolism;
Metabolism > Amino acid metabolism > Glycine cleavage system
spinalcord, liver, adipose, smallintestine, largeintestine, placenta, testis
SIRT5sirtuin ­5SIR2L5, Q9NXA8, gene:2340812literature, APEX_matrix, Rickettsial homolog, mito protein domain, induction, coexpression, MS/MSMatrixMetabolism > Metals and cofactors > NAD biosynthesis and metabolismcerebrum, brainstem, spinalcord, liver, heart, adipose, largeintestine, stomach
GPT2glutamic--pyruvic ­transaminase ­2ALT2, GPT 2, MRT49, Q8TD30, gene:8470612APEX_matrix, targetP signal, Rickettsial homolog, mito protein domain, MS/MS++MatrixMetabolism > Amino acid metabolism > Glutamate metabolismcerebrum, brainstem, liver, skeletalmuscle, adipose, stomach, placenta, testis
TOMM6translocase ­of ­outer ­mitochondrial ­membrane ­6OBTP, TOM6, Q96B49, gene:10018889312GFP, mito protein domain+, coexpression, MS/MS+MOMProtein import, sorting and homeostasis > Protein import and sorting > TOMcerebrum, cerebellum, brainstem, spinalcord, liver, heart, adipose, smallintestine, largeintestine, stomach, placenta, testis
FAM162Afamily ­with ­sequence ­similarity ­162 ­member ­AC3orf28, E2IG5, HGTD-P, Q96A26, gene:2635512APEX_IMS, APEX_matrix, targetP signal, induction, coexpression, MS/MS++MIMnoneall 14
ABCB6ATP ­binding ­cassette ­subfamily ­B ­member ­6 ­(Langereis ­blood ­group)ABC, LAN, MTABC3, PRP, umat, Q9NP58, gene:1005812literature, yeast mito homolog+, Rickettsial homolog, mito protein domain, coexpression, MS/MSMOMMetabolism > Metals and cofactors > Heme synthesis and processing;
Small molecule transport > ABC transporters
cerebrum, brainstem, liver, largeintestine, stomach, placenta, testis
MACROD1mono-ADP ­ribosylhydrolase ­1LRP16, Q9BQ69, gene:2899212APEX_matrix, targetP signal+, coexpression, MS/MS++MatrixSignalingcerebrum, cerebellum, brainstem, spinalcord, liver, heart, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
TSTthiosulfate ­sulfurtransferaseRDS, Q16762, gene:726312literature, APEX_matrix, Rickettsial homolog, mito protein domain, coexpression, MS/MS++MatrixMetabolism > Detoxification > Xenobiotic metabolism;
Metabolism > Sulfur metabolism
all 14
MT-CO1cytochrome ­c ­oxidase ­subunit ­ICOI, MTCO1, P00395, gene:451212literature, APEX_IMS, yeast mito homolog++, Rickettsial homolog, mito protein domain, MS/MSMIMOXPHOS > Complex IV > CIV subunits;
Metabolism > Metals and cofactors > Heme-containing proteins;
OXPHOS > OXPHOS subunits
all 14
MTFR1Lmitochondrial ­fission ­regulator ­1 ­likeFAM54B, HYST1888, MST116, MSTP116, Q9H019, gene:5618112mito protein domain+, induction, coexpression, MS/MSunknownnonecerebrum, cerebellum, brainstem, spinalcord, placenta, testis
SDR39U1short ­chain ­dehydrogenase/reductase ­family ­39U ­member ­1C14orf124, HCDI, Q9NRG7, gene:5694812mito protein domain, coexpression, MS/MS++unknownnonecerebrum, cerebellum, brainstem, spinalcord, kidney, heart, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
DMGDHdimethylglycine ­dehydrogenaseDMGDHD, ME2GLYDH, Q9UI17, gene:2995812literature, targetP signal, mito protein domain, MS/MS++MatrixMetabolism > Amino acid metabolism > Glycine metabolism;
Metabolism > Vitamin metabolism > Choline and betaine metabolism;
Metabolism > Vitamin metabolism > Folate and 1-C metabolism
spinalcord, kidney, liver, smallintestine, stomach, placenta
SLC25A14solute ­carrier ­family ­25 ­member ­14BMCP1, UCP5, O95258, gene:901612literature, yeast mito homolog+, mito protein domain+, MS/MSMIMMetabolism > Sulfur metabolism;
Small molecule transport > SLC25A family
cerebrum, cerebellum, brainstem, placenta, testis
PRELID3APRELI ­domain ­containing ­3AC18orf43, HFL-EDDG1, SLMO1, Q96N28, gene:1065012yeast mito homolog+, mito protein domain+, MS/MSIMSMetabolism > Lipid metabolism > Cardiolipin synthesis;
Metabolism > Lipid metabolism > Phospholipid metabolism
cerebrum, cerebellum, spinalcord
CHCHD5coiled-coil-helix-coiled-coil-helix ­domain ­containing ­5C2orf9, CHTM1, MIC14, MIX14, Q9BSY4, gene:8426912APEX_IMS, yeast mito homolog++, mito protein domain, coexpression, MS/MSIMSnoneadipose, placenta, testis
TOMM70translocase ­of ­outer ­mitochondrial ­membrane ­70TOMM70A, Tom70, O94826, gene:986812literature, yeast mito homolog+, mito protein domain, induction, coexpressionMOMProtein import, sorting and homeostasis > Protein import and sorting > TOMcerebrum, cerebellum, brainstem, spinalcord, kidney, liver, skeletalmuscle, adipose, largeintestine, stomach, placenta, testis
NIT1nitrilase ­1Q86X76, gene:481712APEX_matrix, targetP signal, mito protein domain, coexpression, MS/MS++MatrixMetabolism > Detoxification > ROS and glutathione metabolismcerebrum, cerebellum, brainstem, spinalcord, kidney, liver, adipose, smallintestine, largeintestine, stomach, placenta, testis
FAHD1fumarylacetoacetate ­hydrolase ­domain ­containing ­1C16orf36, YISKL, Q6P587, gene:8188912literature, APEX_matrix, mito protein domain, coexpression, MS/MS++MatrixMetabolism > Carbohydrate metabolism > TCA-associatedall 14
MCUmitochondrial ­calcium ­uniporterC10orf42, CCDC109A, HsMCU, Q8NE86, gene:9055012literature, GFP, mito protein domain+, MS/MS++MIMSignaling > Calcium homeostasis > Calcium cycle;
Small molecule transport > Calcium uniporter
cerebrum, cerebellum, brainstem, spinalcord, liver, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta
CYP27A1cytochrome ­P450 ­family ­27 ­subfamily ­A ­member ­1CP27, CTX, CYP27, Q02318, gene:159312literature, targetP signal+, mito protein domain, MS/MS++MIMMetabolism > Lipid metabolism > Cholesterol, bile acid, steroid synthesis;
Metabolism > Metals and cofactors > Heme-containing proteins
kidney, liver, skeletalmuscle, adipose, smallintestine, placenta, testis
MT-ND2MTND2MTND2, P03891, gene:453612literature, Rickettsial homolog, mito protein domain+, MS/MS+MIMOXPHOS > Complex I > CI subunits;
OXPHOS > OXPHOS subunits
cerebrum, cerebellum, brainstem, spinalcord, liver, heart, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
MT-ND4NADH ­dehydrogenase, ­subunit ­4 ­(complex ­I)MTND4, P03905, gene:453812literature, APEX_IMS, APEX_matrix, Rickettsial homolog, mito protein domain+, MS/MS+MIMOXPHOS > Complex I > CI subunits;
OXPHOS > OXPHOS subunits
all 14
MT-ND5NADH ­dehydrogenase, ­subunit ­5 ­(complex ­I)MTND5, P03915, gene:454012literature, APEX_IMS, APEX_matrix, Rickettsial homolog, mito protein domain+, MS/MS+MIMOXPHOS > Complex I > CI subunits;
OXPHOS > OXPHOS subunits
all 14
DELE1DAP3 ­binding ­cell ­death ­enhancer ­1DELE, DELE1(L), KIAA0141, Q14154, gene:981212targetP signal+, mito protein domain, induction, coexpressionMIMSignalingnone
FASTKD1FAST ­kinase ­domains ­1Q53R41, gene:7967512APEX_matrix, induction, coexpression++MatrixMitochondrial central dogma > mtRNA metabolism > mtRNA granules;
Mitochondrial central dogma > mtRNA metabolism > Polycistronic mtRNA processing;
Mitochondrial central dogma > mtRNA metabolism > mtRNA stability and decay
liver, largeintestine
MRPS31mitochondrial ­ribosomal ­protein ­S31IMOGN38, MRP-S31, S31mt, Q92665, gene:1024012literature, APEX_matrix, targetP signal, induction, coexpression, MS/MSMatrixMitochondrial central dogma > Translation > Mitochondrial ribosomeall 14
ALDH1L1aldehyde ­dehydrogenase ­1 ­family ­member ­L110-FTHFDH, 10-fTHF, FDH, FTHFD, O75891, gene:1084012APEX_matrix, Rickettsial homolog, mito protein domain, induction, coexpression, MS/MSMatrixMetabolism > Vitamin metabolism > Folate and 1-C metabolismcerebrum, liver, smallintestine, largeintestine, stomach, placenta, testis
PTCD1pentatricopeptide ­repeat ­domain ­1O75127, gene:2602411targetP signal+, mito protein domain+, coexpressionMatrixMitochondrial central dogma > mtRNA metabolism > mtRNA granulescerebellum, largeintestine, placenta
HADHBhydroxyacyl-CoA ­dehydrogenase ­trifunctional ­multienzyme ­complex ­subunit ­betaECHB, MSTP029, MTPB, TP-BETA, P55084, gene:303211literature, targetP signal, Rickettsial homolog, mito protein domain, MS/MS++MIMMetabolism > Carbohydrate metabolism > Ketone metabolism;
Metabolism > Lipid metabolism > Fatty acid oxidation;
Metabolism > Amino acid metabolism > Lysine metabolism
all 14
TRIT1tRNA ­isopentenyltransferase ­1COXPD35, GRO1, IPPT, IPT, IPTase, MOD5, hGRO1, Q9H3H1, gene:5480211literature, APEX_matrix, targetP signal, yeast mito homolog, Rickettsial homolog, mito protein domain, coexpressionMatrixMitochondrial central dogma > mtRNA metabolism > mt-tRNA modifications;
Mitochondrial central dogma > mtRNA metabolism > mt-rRNA modifications
cerebellum
CRLS1cardiolipin ­synthase ­1C20orf155, CLS, CLS1, GCD10, dJ967N21.6, Q9UJA2, gene:5467511literature, targetP signal, yeast mito homolog, Rickettsial homolog, mito protein domain, coexpressionMIMMetabolism > Lipid metabolism > Cardiolipin synthesisnone
GLYCTKglycerate ­kinaseHBEBP2, HBEBP4, HBeAgBP4A, Q8IVS8, gene:13215811targetP signal+, coexpression, MS/MS+MatrixMetabolism > Carbohydrate metabolismkidney, liver, placenta
SLC25A32solute ­carrier ­family ­25 ­member ­32MFT, MFTC, RREI, Q9H2D1, gene:8103411literature, targetP signal, yeast mito homolog+, mito protein domain+MIMMetabolism > Vitamin metabolism > Vitamin B2 metabolism;
Metabolism > Vitamin metabolism > Folate and 1-C metabolism;
Small molecule transport > SLC25A family
adipose, placenta
AARS2alanyl-tRNA ­synthetase ­2, ­mitochondrialAARSL, COXPD8, LKENP, MT-ALARS, MTALARS, Q5JTZ9, gene:5750511literature, APEX_matrix, targetP signal+, Rickettsial homolog, mito protein domain, MS/MSMatrixMitochondrial central dogma > Translation > mt-tRNA synthetasescerebrum, cerebellum, brainstem, spinalcord, kidney, liver, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
EXOGexo/endonuclease ­GENDOGL1, ENDOGL2, ENGL, ENGL-a, ENGL-b, ENGLA, ENGLB, Q9Y2C4, gene:994111APEX_IMS, targetP signal, mito protein domain, MS/MS++MIMMitochondrial central dogma > mtDNA maintenance > mtDNA replication;
Mitochondrial central dogma > mtDNA maintenance > mtDNA repair;
Mitochondrial central dogma > mtDNA maintenance > mtDNA stability and decay
cerebrum, cerebellum, brainstem, spinalcord, adipose, largeintestine, placenta
CHCHD2coiled-coil-helix-coiled-coil-helix ­domain ­containing ­2C7orf17, MIX17B, MNRR1, NS2TP, PARK22, Q9Y6H1, gene:5114211APEX_IMS, targetP signal, yeast mito homolog+, mito protein domain, coexpressionIMSMitochondrial dynamics and surveillance > Apoptosisnone
KMOkynurenine ­3-monooxygenasedJ317G22.1, O15229, gene:856411yeast mito homolog, Rickettsial homolog, mito protein domain, MS/MS++MOMMetabolism > Amino acid metabolism > Kynurenine metabolism;
Metabolism > Metals and cofactors > NAD biosynthesis and metabolism
kidney, liver
SFXN5sideroflexin ­5BBG-TCC, SLC56A5, Q8TD22, gene:9409711literature, mito protein domain+, MS/MS++MIMMetabolism > Carbohydrate metabolism > TCA-associated;
Small molecule transport > Sideroflexins
cerebrum, cerebellum, brainstem, spinalcord, kidney, liver, adipose, largeintestine
ELAC2elaC ­ribonuclease ­Z ­2COXPD17, ELC2, HPC2, Q9BQ52, gene:6052811APEX_matrix, GFP, targetP signal, Rickettsial homolog, mito protein domain, MS/MSMatrixMitochondrial central dogma > mtRNA metabolism > Polycistronic mtRNA processingcerebellum, skeletalmuscle, adipose, largeintestine, stomach
GPX1glutathione ­peroxidase ­1GPXD, GSHPX1, P07203, gene:287611literature, APEX_matrix, targetP signal, mito protein domain, coexpression, MS/MS+MatrixMetabolism > Detoxification > ROS and glutathione metabolism;
Metabolism > Detoxification > Selenoproteins
cerebrum, cerebellum, brainstem, spinalcord, kidney, liver, adipose, smallintestine, largeintestine, stomach, placenta, testis
CPT1Bcarnitine ­palmitoyltransferase ­1BCPT1-M, CPT1M, CPTI, CPTI-M, M-CPT1, MCCPT1, MCPT1, Q92523, gene:137511literature, yeast mito homolog+, mito protein domain, MS/MS++MOMMetabolism > Lipid metabolism > Fatty acid oxidation;
Metabolism > Metals and cofactors > Carnitine synthesis and transport;
Metabolism > Metals and cofactors > Carnitine shuttle
cerebellum, brainstem, spinalcord, kidney, liver, heart, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
FIS1fission, ­mitochondrial ­1CGI-135, TTC11, Q9Y3D6, gene:5102411literature, yeast mito homolog++, mito protein domain+MOMMitochondrial dynamics and surveillance > Fissionall 14
PRELID1PRELI ­domain ­containing ­1CGI-106, PRELI, PX19, SBBI12, Q9Y255, gene:2716611literature, APEX_IMS, GFP, targetP signal, yeast mito homolog++, mito protein domain+IMSMetabolism > Lipid metabolism > Cardiolipin synthesis;
Metabolism > Lipid metabolism > Phospholipid metabolism
none
UCP3uncoupling ­protein ­3SLC25A9, P55916, gene:735211literature, yeast mito homolog+, mito protein domain+, MS/MSMIMSmall molecule transport > SLC25A familyspinalcord, heart, skeletalmuscle, adipose
SLC25A27solute ­carrier ­family ­25 ­member ­27UCP4, O95847, gene:948111literature, yeast mito homolog+, mito protein domain+, MS/MSMIMSmall molecule transport > SLC25A familycerebrum, cerebellum, brainstem, spinalcord
SLC25A40solute ­carrier ­family ­25 ­member ­40MCFP, Q8TBP6, gene:5597211literature, yeast mito homolog+, mito protein domain+, MS/MSMIMSmall molecule transport > SLC25A familycerebrum, cerebellum, brainstem, spinalcord, adipose, largeintestine, stomach, placenta, testis
SLC25A23solute ­carrier ­family ­25 ­member ­23APC2, MCSC2, SCaMC-3, Q9BV35, gene:7908511literature, yeast mito homolog+, mito protein domain+, MS/MSMIMMetabolism > Nucleotide metabolism > Nucleotide import;
Signaling > Calcium homeostasis > EF hand proteins;
Small molecule transport > SLC25A family
cerebrum, brainstem, spinalcord, liver, adipose, smallintestine, largeintestine, stomach, placenta
HEMK1HemK ­methyltransferase ­family ­member ­1HEMK, MPRMC, MTQ1, Q9Y5R4, gene:5140911GFP, targetP signal, yeast mito homolog, Rickettsial homolog, mito protein domain+MatrixMitochondrial central dogma > Translation > Translation factorsnone
SLC25A36solute ­carrier ­family ­25 ­member ­36PNC2, Q96CQ1, gene:5518611GFP, yeast mito homolog+, mito protein domain+, coexpressionMIMMetabolism > Nucleotide metabolism > Nucleotide import;
Small molecule transport > SLC25A family
brainstem, spinalcord, placenta, testis
OMA1OMA1 ­zinc ­metallopeptidase2010001O09Rik, DAB1, MPRP-1, MPRP1, YKR087C, ZMPOMA1, peptidase, Q96E52, gene:11520911APEX_IMS, GFP, targetP signal+, yeast mito homolog++, coexpressionMIMProtein import, sorting and homeostasis > Protein homeostasis > Proteases;
Mitochondrial dynamics and surveillance > Fusion;
Mitochondrial dynamics and surveillance > Fission
skeletalmuscle, adipose, largeintestine
LETM2leucine ­zipper ­and ­EF-hand ­containing ­transmembrane ­protein ­2SLC55A2, Q2VYF4, gene:13799411targetP signal, yeast mito homolog+, mito protein domain+MIMnonenone
DHRS1dehydrogenase/reductase ­1SDR19C1, Q96LJ7, gene:11581711Rickettsial homolog, mito protein domain, MS/MS++MIMMetabolism > Lipid metabolism > Cholesterol, bile acid, steroid synthesiscerebrum, cerebellum, brainstem, spinalcord, kidney, liver, adipose, smallintestine, largeintestine, stomach, placenta, testis
STOMstomatinBND7, EPB7, EPB72, P27105, gene:204011yeast mito homolog+, Rickettsial homolog, mito protein domain, MS/MSMembranenoneadipose, placenta, testis
METTL5methyltransferase ­like ­5HSPC133, MRT72, Q9NRN9, gene:2908110Rickettsial homolog, mito protein domain+, induction, coexpressionMatrixMitochondrial central dogma > mtRNA metabolism > mt-rRNA modificationsnone
MSRAmethionine ­sulfoxide ­reductase ­APMSR, Q9UJ68, gene:448210mito protein domain, coexpression, MS/MS++MatrixMetabolism > Detoxification > ROS and glutathione metabolism;
Metabolism > Sulfur metabolism
cerebrum, cerebellum, brainstem, spinalcord, kidney, liver, heart, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta
NUDT8nudix ­hydrolase ­8Q8WV74, gene:25455210APEX_matrix, mito protein domain, coexpression, MS/MS++MatrixMetabolism > Metals and cofactors > Coenzyme A metabolismcerebrum, cerebellum, brainstem, spinalcord, kidney, liver, heart, skeletalmuscle, adipose, largeintestine, stomach, placenta, testis
NMNAT3nicotinamide ­nucleotide ­adenylyltransferase ­3FKSG76, PNAT-3, PNAT3, Q96T66, gene:34956510literature, mito protein domain, coexpression, MS/MS++MatrixMetabolism > Metals and cofactors > NAD biosynthesis and metabolismcerebrum, cerebellum, brainstem, spinalcord, kidney, liver, heart, skeletalmuscle, adipose, smallintestine, largeintestine, placenta, testis
SLC25A38solute ­carrier ­family ­25 ­member ­38SIDBA2, Q96DW6, gene:5497710yeast mito homolog++, mito protein domain+, coexpressionMIMMetabolism > Amino acid metabolism > Glycine metabolism;
Metabolism > Metals and cofactors > Heme synthesis and processing;
Metabolism > Vitamin metabolism > Folate and 1-C metabolism;
Small molecule transport > SLC25A family
none
MPV17L2MPV17 ­mitochondrial ­inner ­membrane ­protein ­like ­2FKSG24, Q567V2, gene:8476910GFP, targetP signal, yeast mito homolog+, mito protein domain, coexpressionMIMMitochondrial central dogma > Translation > Mitochondrial ribosome assemblylargeintestine, placenta
ACLYATP ­citrate ­lyaseACL, ATPCL, CLATP, P53396, gene:4710APEX_matrix, yeast mito homolog+, Rickettsial homolog, mito protein domain, coexpressionMatrixMetabolism > Carbohydrate metabolism > TCA-associatedcerebrum, cerebellum, brainstem, spinalcord, kidney, liver, adipose, largeintestine, stomach, placenta, testis
ABCD2ATP ­binding ­cassette ­subfamily ­D ­member ­2ABC39, ALDL1, ALDR, ALDRP, hALDR, Q9UBJ2, gene:22510targetP signal, Rickettsial homolog, mito protein domain, coexpression, MS/MS+MembraneSmall molecule transport > ABC transportersadipose
FLAD1flavin ­adenine ­dinucleotide ­synthetase ­1FAD1, FADS, LSMFLAD, PP591, Q8NFF5, gene:8030810APEX_matrix, targetP signal, mito protein domain, induction, coexpressionMatrixMetabolism > Vitamin metabolism > Vitamin B2 metabolismspinalcord, adipose, stomach
LIPT1lipoyltransferase ­1LIPT1D, Q9Y234, gene:5160110GFP, yeast mito homolog++, mito protein domain, coexpressionMatrixMetabolism > Lipid metabolism > Lipoate insertionadipose, placenta
DGLUCYD-glutamate ­cyclaseC14orf159, Q7Z3D6, gene:8001710literature, APEX_matrix, targetP signal, coexpression, MS/MS++MatrixMetabolism > Amino acid metabolismall 14
PCK2phosphoenolpyruvate ­carboxykinase ­2, ­mitochondrialPEPCK, PEPCK-M, PEPCK2, Q16822, gene:510610literature, targetP signal, mito protein domain, coexpression, MS/MS++MatrixMetabolism > Carbohydrate metabolism > Gluconeogenesis;
Metabolism > Carbohydrate metabolism > TCA-associated
cerebrum, cerebellum, brainstem, spinalcord, adipose, smallintestine, largeintestine, stomach, placenta, testis
NADK2NAD ­kinase ­2, ­mitochondrialC5orf33, DECRD, MNADK, NADKD1, Q4G0N4, gene:13368610APEX_matrix, targetP signal, mito protein domain, coexpression, MS/MS++MatrixMetabolism > Metals and cofactors > NAD biosynthesis and metabolismcerebrum, cerebellum, brainstem, spinalcord, kidney, liver, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
MPSTmercaptopyruvate ­sulfurtransferaseMST, TST2, TUM1, P25325, gene:435710APEX_matrix, mito protein domain, coexpression, MS/MS++MIMMetabolism > Detoxification > Xenobiotic metabolism;
Metabolism > Sulfur metabolism
cerebrum, cerebellum, brainstem, spinalcord, kidney, liver, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
NUDT13nudix ­hydrolase ­13Q86X67, gene:2596110GFP, targetP signal, mito protein domain, induction, coexpression, MS/MSMatrixMetabolism > Metals and cofactors > NAD biosynthesis and metabolismcerebellum, brainstem, heart, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta
ACACAacetyl-CoA ­carboxylase ­alphaACAC, ACACAD, ACC, ACC1, ACCA, Q13085, gene:3110targetP signal, Rickettsial homolog, mito protein domain, coexpressionMatrixMetabolism > Lipid metabolism > Type II fatty acid synthesis;
Metabolism > Vitamin metabolism > Biotin utilizing proteins
brainstem, spinalcord, liver, adipose, largeintestine, stomach, placenta, testis
ABAT4-aminobutyrate ­aminotransferaseGABA-AT, GABAT, NPD009, P80404, gene:1810literature, APEX_matrix, targetP signal, mito protein domain, MS/MS++MatrixMetabolism > Amino acid metabolism > GABA metabolism;
Metabolism > Metals and cofactors > Fe-S-containing proteins
cerebrum, cerebellum, brainstem, spinalcord, kidney, liver, heart, adipose, smallintestine, largeintestine, stomach, placenta, testis
HMGCS23-hydroxy-3-methylglutaryl-CoA ­synthase ­2P54868, gene:315810literature, targetP signal, mito protein domain, MS/MS++MatrixMetabolism > Carbohydrate metabolism > Ketone metabolismcerebellum, spinalcord, liver, adipose, smallintestine, largeintestine, stomach, placenta, testis
ALDH5A1aldehyde ­dehydrogenase ­5 ­family ­member ­A1SSADH, SSDH, P51649, gene:791510APEX_matrix, targetP signal, mito protein domain, MS/MS++MatrixMetabolism > Amino acid metabolism > GABA metabolismall 14
CKMT1Acreatine ­kinase, ­mitochondrial ­1ACKMT1, U-MtCK, mia-CK, 0, gene:54859610literature, targetP signal, mito protein domain, MS/MS++MIMMetabolism > Nucleotide metabolism > Creatine metabolismcerebrum, cerebellum, brainstem, spinalcord, kidney, heart, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta
PISDphosphatidylserine ­decarboxylaseDJ858B16, LIBF, PSD, PSDC, PSSC, dJ858B16.2, Q9UG56, gene:2376110yeast mito homolog++, mito protein domain, MS/MSMIMMetabolism > Lipid metabolism > Phospholipid metabolismcerebrum, cerebellum, brainstem, spinalcord, kidney, liver, adipose, smallintestine, largeintestine, stomach, placenta, testis
NDUFB4NADH:ubiquinone ­oxidoreductase ­subunit ­B4B15, CI-B15, O95168, gene:471010literature, mito protein domain+, MS/MS++MIMOXPHOS > Complex I > CI subunits;
OXPHOS > OXPHOS subunits
all 14
UQCR11ubiquinol-cytochrome ­c ­reductase, ­complex ­III ­subunit ­XI0710008D09Rik, QCR10, UQCR, O14957, gene:1097510literature, mito protein domain+, MS/MS++MIMOXPHOS > Complex III > CIII subunits;
OXPHOS > OXPHOS subunits
cerebrum, cerebellum, brainstem, spinalcord, kidney, liver, heart, adipose, smallintestine, stomach, placenta, testis
FAM136Afamily ­with ­sequence ­similarity ­136 ­member ­AQ96C01, gene:8490810APEX_IMS, mito protein domain+, MS/MS++IMSnoneall 14
TOMM5translocase ­of ­outer ­mitochondrial ­membrane ­5C9orf105, Tom5, bA613M10.3, Q8N4H5, gene:40150510literature, mito protein domain+, MS/MS++MOMProtein import, sorting and homeostasis > Protein import and sorting > TOMcerebrum, cerebellum, brainstem, spinalcord, kidney, liver, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
HTRA2HtrA ­serine ­peptidase ­2MGCA8, OMI, PARK13, PRSS25, O43464, gene:2742910literature, APEX_IMS, targetP signal, Rickettsial homolog, mito protein domain, MS/MS+MIMProtein import, sorting and homeostasis > Protein homeostasis > Proteases;
Mitochondrial dynamics and surveillance > Apoptosis
cerebrum, cerebellum, brainstem, spinalcord, kidney, liver, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
NDUFA1NADH:ubiquinone ­oxidoreductase ­subunit ­A1CI-MWFE, MC1DN12, MWFE, ZNF183, O15239, gene:469410literature, coexpression+, MS/MS++MIMOXPHOS > Complex I > CI subunits;
OXPHOS > OXPHOS subunits
all 14
MT-CYBcytochrome ­bMTCYB, P00156, gene:451910literature, yeast mito homolog++, Rickettsial homolog, mito protein domainMIMOXPHOS > Complex III > CIII subunits;
Metabolism > Metals and cofactors > Heme-containing proteins;
Metabolism > Electron carriers > Cytochromes;
OXPHOS > OXPHOS subunits
all 14
GUK1guanylate ­kinase ­1GMK, Q16774, gene:298710APEX_matrix, targetP signal, Rickettsial homolog, induction, coexpression, MS/MSMatrixMetabolism > Nucleotide metabolism > Nucleotide synthesis and processingcerebrum, cerebellum, brainstem, spinalcord, adipose, largeintestine, stomach
D2HGDHD-2-hydroxyglutarate ­dehydrogenaseD2HGD, Q8N465, gene:72829410literature, APEX_matrix, targetP signal, yeast mito homolog++, mito protein domainMatrixMetabolism > Carbohydrate metabolism > TCA-associatedadipose, largeintestine
OPA3outer ­mitochondrial ­membrane ­lipid ­metabolism ­regulator ­OPA3MGA3, Q9H6K4, gene:8020710literature, targetP signal, coexpression, MS/MS++MOMnoneall 14
AKR1B10aldo-keto ­reductase ­family ­1 ­member ­B10AKR1B11, AKR1B12, ALDRLn, ARL-1, ARL1, HIS, HSI, O60218, gene:5701610induction, coexpression, MS/MS++unknownMetabolism > Vitamin metabolism > Vitamin A metabolism;
Metabolism > Detoxification > Xenobiotic metabolism
cerebrum, brainstem, spinalcord, kidney, heart, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
PHYHphytanoyl-CoA ­2-hydroxylaseLN1, LNAP1, PAHX, PHYH1, RD, O14832, gene:526410targetP signal, induction, MS/MS++MatrixMetabolism > Lipid metabolismkidney, liver, smallintestine
NDUFAF2NADH:ubiquinone ­oxidoreductase ­complex ­assembly ­factor ­2B17.2L, MC1DN10, MMTN, NDUFA12L, mimitin, Q8N183, gene:9194210literature, APEX_matrix, induction, coexpression, MS/MS++MIMOXPHOS > Complex I > CI assembly factors;
OXPHOS > OXPHOS assembly factors
all 14
ECSITECSIT ­signaling ­integratorSITPEC, Q9BQ95, gene:5129510literature, APEX_matrix, targetP signal, mito protein domain, induction, coexpression+MIMOXPHOS > Complex I > CI assembly factors;
OXPHOS > OXPHOS assembly factors
brainstem, spinalcord, kidney, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
PARLpresenilin ­associated ­rhomboid ­likePRO2207, PSARL, PSARL1, PSENIP2, RHBDS1, Q9H300, gene:5548610literature, yeast mito homolog++, mito protein domain, MS/MSMIMProtein import, sorting and homeostasis > Protein homeostasis > Proteases;
Mitochondrial dynamics and surveillance > Mitophagy;
Mitochondrial dynamics and surveillance > Autophagy
cerebrum, cerebellum, brainstem, spinalcord, kidney, liver, heart, adipose, smallintestine, largeintestine, stomach, placenta, testis
MRPL42mitochondrial ­ribosomal ­protein ­L42HSPC204, L31MT, L42MT, MRP-L31, MRP-L42, MRP-S32, MRPL31, MRPS32, PTD007, RPML31, S32MT, Q9Y6G3, gene:2897710APEX_matrix, GFP, mito protein domain+, induction, MS/MSMatrixMitochondrial central dogma > Translation > Mitochondrial ribosomeliver, adipose, largeintestine, testis
DNAJC4DnaJ ­heat ­shock ­protein ­family ­(Hsp40) ­member ­C4DANJC4, HSPF2, MCG18, Q9NNZ3, gene:333810GFP, targetP signal+, Rickettsial homolog, mito protein domain, coexpressionMembraneProtein import, sorting and homeostasis > Protein homeostasislargeintestine
PAM16presequence ­translocase ­associated ­motor ­16CGI-136, MAGMAS, SMDMDM, TIM16, TIMM16, Q9Y3D7, gene:5102510literature, APEX_matrix, yeast mito homolog, MS/MS++MIMProtein import, sorting and homeostasis > Protein import and sorting > Import motorall 14
ALDH3A2aldehyde ­dehydrogenase ­3 ­family ­member ­A2ALDH10, FALDH, SLS, P51648, gene:22410targetP signal+, mito protein domain, coexpressionMembraneMetabolism > Detoxification > ROS and glutathione metabolismspinalcord, kidney, liver, smallintestine, largeintestine, stomach, placenta, testis
ACSL6acyl-CoA ­synthetase ­long ­chain ­family ­member ­6ACS2, FACL6, LACS 6, LACS2, LACS5, Q9UKU0, gene:2330510Rickettsial homolog, mito protein domain, MS/MS++MOMMetabolism > Lipid metabolism > Fatty acid oxidationcerebrum, cerebellum, brainstem, spinalcord, liver, heart, skeletalmuscle, adipose, largeintestine, stomach, testis
MRS2magnesium ­transporter ­MRS2HPT, MRS2L, Q9HD23, gene:5738010APEX_matrix, GFP, targetP signal, yeast mito homolog++, coexpressionMIMSmall molecule transport;
Signaling
cerebrum, cerebellum, brainstem, spinalcord, skeletalmuscle, smallintestine, largeintestine, stomach, placenta, testis
ABHD10abhydrolase ­domain ­containing ­10, ­depalmitoylaseQ9NUJ1, gene:553479literature, APEX_IMS, APEX_matrix, Rickettsial homolog, mito protein domain, MS/MS++MatrixMetabolism > Detoxification > Xenobiotic metabolismcerebrum, cerebellum, brainstem, spinalcord, liver, adipose, smallintestine, largeintestine, stomach, placenta, testis
HINT3histidine ­triad ­nucleotide ­binding ­protein ­3HINT4, Q9NQE9, gene:1351149Rickettsial homolog, mito protein domain, induction, coexpression+unknownnonebrainstem, heart, skeletalmuscle
PXMP4peroxisomal ­membrane ­protein ­4PMP24, Q9Y6I8, gene:112649targetP signal, mito protein domain+, coexpressionunknownnonekidney, liver, adipose
OSGEPL1O-sialoglycoprotein ­endopeptidase ­like ­1OSGEPL, Qri7, Q9H4B0, gene:641729APEX_matrix, targetP signal, yeast mito homolog, Rickettsial homolog, mito protein domain, coexpressionMatrixMitochondrial central dogma > mtRNA metabolism > mt-tRNA modificationsnone
NUDT2nudix ­hydrolase ­2APAH1, P50583, gene:3189mito protein domain, coexpression, MS/MS++MatrixMetabolism > Nucleotide metabolism > Nucleotide synthesis and processingall 14
CYB5Bcytochrome ­b5 ­type ­BCYB5-M, CYPB5M, OMB5, O43169, gene:807779literature, mito protein domain, coexpression, MS/MS++MOMMetabolism > Metals and cofactors > Heme-containing proteins;
Metabolism > Detoxification > Xenobiotic metabolism;
Metabolism > Detoxification > Amidoxime reducing complex;
Metabolism > Electron carriers > Cytochromes
all 14
GRSF1G-rich ­RNA ­sequence ­binding ­factor ­1Q12849, gene:29269APEX_matrix, mito protein domain, induction, coexpression, MS/MSMatrixMitochondrial central dogma > mtRNA metabolism > mtRNA granules;
Mitochondrial central dogma > mtRNA metabolism > Polycistronic mtRNA processing;
Mitochondrial central dogma > mtRNA metabolism > mtRNA stability and decay;
Mitochondrial central dogma > Translation > Mitochondrial ribosome assembly
adipose, smallintestine, largeintestine, stomach, placenta, testis
MRPS18Bmitochondrial ­ribosomal ­protein ­S18BC6orf14, HSPC183, HumanS18a, MRP-S18-2, MRPS18-2, PTD017, S18amt, Q9Y676, gene:289739literature, APEX_matrix, targetP signal+, Rickettsial homolog, mito protein domain, MS/MSMatrixMitochondrial central dogma > Translation > Mitochondrial ribosomecerebrum, cerebellum, brainstem, spinalcord, liver, heart, adipose, smallintestine, largeintestine, placenta, testis
DNAJC30DnaJ ­heat ­shock ­protein ­family ­(Hsp40) ­member ­C30WBSCR18, Q96LL9, gene:842779APEX_matrix, GFP, targetP signal+, Rickettsial homolog, mito protein domain, MS/MSMIMnonecerebrum, cerebellum, brainstem, spinalcord, heart, skeletalmuscle, adipose, largeintestine, stomach
CATcatalaseP04040, gene:8479yeast mito homolog++, mito protein domain, coexpressionIMSMetabolism > Detoxification > ROS and glutathione metabolismspinalcord, kidney, liver, heart, adipose, smallintestine, largeintestine, stomach, placenta, testis
DGUOKdeoxyguanosine ­kinaseMTDPS3, NCPH, PEOB4, dGK, Q16854, gene:17169literature, APEX_matrix, targetP signal, mito protein domain, MS/MS++MatrixMetabolism > Nucleotide metabolism > Nucleotide synthesis and processingcerebrum, cerebellum, brainstem, spinalcord, liver, heart, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
ACOT9acyl-CoA ­thioesterase ­9ACATE2, CGI-16, MT-ACT48, MTACT48, Q9Y305, gene:235979literature, targetP signal, mito protein domain, MS/MS++MIMMetabolism > Lipid metabolismcerebrum, cerebellum, brainstem, spinalcord, kidney, heart, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
ACSS1acyl-CoA ­synthetase ­short ­chain ­family ­member ­1ACAS2L, ACECS1, AceCS2L, Q9NUB1, gene:845329literature, APEX_matrix, targetP signal, mito protein domain, MS/MS++MIMMetabolism > Lipid metabolism > Fatty acid oxidationcerebrum, cerebellum, brainstem, spinalcord, kidney, heart, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
RFKriboflavin ­kinaseRIFK, Q969G6, gene:553129targetP signal, yeast mito homolog++, MS/MSMatrixMetabolism > Vitamin metabolism > Vitamin B2 metabolismbrainstem, spinalcord, kidney, adipose, smallintestine, largeintestine
STARD7StAR ­related ­lipid ­transfer ­domain ­containing ­7FAME2, GTT1, Q9NQZ5, gene:569109APEX_IMS, targetP signal, mito protein domain, induction, coexpression+IMSMetabolism > Lipid metabolism > Phospholipid metabolism;
Small molecule transport
none
CHPT1choline ­phosphotransferase ­1CPT, CPT1, Q8WUD6, gene:569949targetP signal, mito protein domain, induction, coexpression+MIMMetabolism > Lipid metabolism > Phospholipid metabolismnone
MT-ATP8ATP ­synthase ­F0 ­subunit ­8ATPase8, MTATP8, P03928, gene:45099literature, mito protein domain+, MS/MS++MIMOXPHOS > Complex V > CV subunits;
OXPHOS > OXPHOS subunits
all 14
TIMMDC1translocase ­of ­inner ­mitochondrial ­membrane ­domain ­containing ­1C3orf1, MC1DN31, Q9NPL8, gene:513009literature, APEX_IMS, APEX_matrix, mito protein domain+, MS/MS++MIMOXPHOS > Complex I > CI assembly factors;
OXPHOS > OXPHOS assembly factors
cerebrum, cerebellum, brainstem, spinalcord, liver, heart, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
SPATA19spermatogenesis ­associated ­19CT132, SPAS1, spergen1, Q7Z5L4, gene:2199389mito protein domain+, MS/MS++unknownnonetestis
HTATIP2HIV-1 ­Tat ­interactive ­protein ­2CC3, SDR44U1, TIP30, Q9BUP3, gene:105539yeast mito homolog, mito protein domain+, MS/MSMembranenonecerebrum, cerebellum, brainstem, liver, adipose, placenta, testis
AKR7A2aldo-keto ­reductase ­family ­7 ­member ­A2AFAR, AFAR1, AFB1-AR1, AKR7, O43488, gene:85749APEX_matrix, targetP signal, coexpression, MS/MS++MatrixMetabolism > Amino acid metabolism > GABA metabolism;
Metabolism > Detoxification > Xenobiotic metabolism
all 14
DUTdeoxyuridine ­triphosphatasedUTPase, P33316, gene:18549literature, targetP signal, Rickettsial homolog, MS/MS++MatrixMetabolism > Nucleotide metabolism > Nucleotide synthesis and processingcerebrum, spinalcord, liver, heart, adipose, largeintestine, stomach, placenta
MT-CO3cytochrome ­c ­oxidase ­IIICOIII, MTCO3, P00414, gene:45149literature, yeast mito homolog++, Rickettsial homolog, mito protein domainMIMOXPHOS > Complex IV > CIV subunits;
OXPHOS > OXPHOS subunits
all 14
DHRS7Bdehydrogenase/reductase ­7BCGI-93, SDR32C1, Q6IAN0, gene:259799Rickettsial homolog, mito protein domain, induction, coexpressionMembranenonecerebrum, cerebellum, brainstem, spinalcord, kidney, liver, adipose, smallintestine, largeintestine, stomach, placenta, testis
MTRF1mitochondrial ­translation ­release ­factor ­1MRF1, MTTRF1, RF1, O75570, gene:96179literature, APEX_matrix, targetP signal, yeast mito homolog+, Rickettsial homolog, mito protein domainMatrixMitochondrial central dogma > Translation > Translation factorsadipose
TMEM143transmembrane ­protein ­143Q96AN5, gene:552609targetP signal, coexpression+, MS/MSMIMnonecerebrum, cerebellum, brainstem, spinalcord, kidney, liver, heart, skeletalmuscle, adipose, largeintestine, stomach, placenta
NT5M5',3'-nucleotidase, ­mitochondrialdNT-2, dNT2, mdN, Q9NPB1, gene:569539literature, targetP signal, mito protein domain, induction, MS/MSMatrixMetabolism > Nucleotide metabolism > Nucleotide synthesis and processingcerebrum, cerebellum, brainstem, spinalcord, skeletalmuscle, adipose, testis
RHOT1ras ­homolog ­family ­member ­T1ARHT1, MIRO-1, MIRO1, Q8IXI2, gene:552889literature, mito protein domain+, MS/MS+MOMSignaling > Calcium homeostasis > EF hand proteins;
Mitochondrial dynamics and surveillance > Trafficking
all 14
NME4NME/NM23 ­nucleoside ­diphosphate ­kinase ­4NDPK-D, NM23H4, nm23-H4, O00746, gene:48339APEX_matrix, GFP, targetP signal, Rickettsial homolog, mito protein domain, MS/MSMIMMetabolism > Nucleotide metabolism > Nucleotide synthesis and processingadipose, largeintestine, placenta, testis
MTHFSmethenyltetrahydrofolate ­synthetaseHsT19268, NEDMEHM, P49914, gene:105889targetP signal, Rickettsial homolog, mito protein domain, MS/MSMatrixMetabolism > Vitamin metabolism > Folate and 1-C metabolismcerebrum, kidney, liver, adipose, smallintestine, largeintestine, stomach, placenta
TIMM29translocase ­of ­inner ­mitochondrial ­membrane ­29C19orf52, TIM29, Q9BSF4, gene:905809targetP signal+, coexpression, MS/MSMIMProtein import, sorting and homeostasis > Protein import and sorting > TIM22 carrier pathwaycerebrum, cerebellum, brainstem, spinalcord, liver, heart, adipose, largeintestine, placenta, testis
PNPOpyridoxamine ­5'-phosphate ­oxidaseHEL-S-302, PDXPO, Q9NVS9, gene:551639APEX_matrix, yeast mito homolog++, coexpression, MS/MSMatrixMetabolism > Vitamin metabolism > Vitamin B6 metabolismbrainstem, liver
MICU2mitochondrial ­calcium ­uptake ­21110008L20Rik, EFHA1, Q8IYU8, gene:2211549literature, APEX_IMS, GFP, targetP signal+, mito protein domain, MS/MS+MIMSignaling > Calcium homeostasis > Calcium cycle;
Signaling > Calcium homeostasis > EF hand proteins;
Small molecule transport > Calcium uniporter
smallintestine, largeintestine, placenta, testis
PCBD2pterin-4 ­alpha-carbinolamine ­dehydratase ­2DCOH2, DCOHM, PHS2, Q9H0N5, gene:841059targetP signal+, induction, MS/MSunknownMetabolism > Metals and cofactors > Tetrahydobiopterin synthesiscerebrum, cerebellum, brainstem, spinalcord, kidney, liver, adipose, smallintestine, largeintestine, stomach, placenta, testis
QRSL1glutaminyl-tRNA ­amidotransferase ­subunit ­QRSL1COXPD40, GatA, Q9H0R6, gene:552789literature, APEX_matrix, GFP, yeast mito homolog, Rickettsial homolog, mito protein domain, coexpression, MS/MSMatrixMitochondrial central dogma > Translation > mt-tRNA synthetasesbrainstem, heart, skeletalmuscle, adipose, largeintestine, stomach, placenta, testis
ADCK1aarF ­domain ­containing ­kinase ­1Q86TW2, gene:571439yeast mito homolog+, Rickettsial homolog, mito protein domain, MS/MSunknownnonecerebrum, spinalcord, kidney, heart, skeletalmuscle, adipose, stomach, placenta
PRDX6peroxiredoxin ­61-Cys, AOP2, HEL-S-128m, NSGPx, PRX, aiPLA2, p29, P30041, gene:95889yeast mito homolog++, Rickettsial homolog, mito protein domainunknownMetabolism > Lipid metabolism > Phospholipid metabolism;
Metabolism > Detoxification > ROS and glutathione metabolism
cerebrum, cerebellum, brainstem, spinalcord, liver, adipose, smallintestine, largeintestine, placenta, testis
NIPSNAP1nipsnap ­homolog ­1Q9BPW8, gene:85089APEX_matrix, targetP signal, coexpression, MS/MS++MatrixMitochondrial dynamics and surveillance > Mitophagy;
Mitochondrial dynamics and surveillance > Autophagy
all 14
GARS1glycyl-tRNA ­synthetase ­1CMT2D, DSMAV, GARS, GlyRS, HMN5, SMAD1, P41250, gene:26179targetP signal, yeast mito homolog, mito protein domain, inductionMatrixMitochondrial central dogma > Translation > mt-tRNA synthetasescerebrum, cerebellum, brainstem, spinalcord, liver, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
ACSM2Aacyl-CoA ­synthetase ­medium ­chain ­family ­member ­2AA-923A4.1, ACSM2, Q08AH3, gene:1238769targetP signal, mito protein domain, MS/MS+MatrixMetabolism > Lipid metabolism > Fatty acid oxidation;
Metabolism > Detoxification > Xenobiotic metabolism
kidney, liver, heart
SLC25A33solute ­carrier ­family ­25 ­member ­33BMSC-MCP, PNC1, Q9BSK2, gene:842759literature, yeast mito homolog+, mito protein domain+MIMMetabolism > Nucleotide metabolism > Nucleotide import;
Small molecule transport > SLC25A family
brainstem, spinalcord, placenta, testis
LYRM9LYR ­motif ­containing ­9C17orf108, HSD24, A8MSI8, gene:2012299targetP signal, mito protein domain+, MS/MSunknownnonecerebellum, heart, adipose, testis
RAB24RAB24, ­member ­RAS ­oncogene ­familyQ969Q5, gene:539179mito protein domain, induction, coexpression, MS/MSMOMMitochondrial dynamics and surveillance > Fission;
Mitochondrial dynamics and surveillance > Autophagy
cerebrum, cerebellum, brainstem, spinalcord, skeletalmuscle, adipose, stomach, testis
PARS2prolyl-tRNA ­synthetase ­2, ­mitochondrialEIEE75, MT-PRORS, proRS, Q7L3T8, gene:259738APEX_matrix, GFP, targetP signal, yeast mito homolog, Rickettsial homolog, mito protein domainMatrixMitochondrial central dogma > Translation > mt-tRNA synthetasessmallintestine, largeintestine, stomach
MRPL52mitochondrial ­ribosomal ­protein ­L52Q86TS9, gene:1227048literature, APEX_matrix, targetP signal, coexpression, MS/MSMatrixMitochondrial central dogma > Translation > Mitochondrial ribosomecerebrum, spinalcord, liver, heart, adipose, smallintestine, largeintestine, stomach, placenta, testis
PUS1pseudouridine ­synthase ­1MLASA1, Q9Y606, gene:803248APEX_matrix, targetP signal, yeast mito homolog+, mito protein domain, coexpressionMatrixMitochondrial central dogma > mtRNA metabolism > mtRNA granules;
Mitochondrial central dogma > mtRNA metabolism > mt-tRNA modifications
none
GOT2glutamic-oxaloacetic ­transaminase ­2KAT4, KATIV, KYAT4, mitAAT, P00505, gene:28068literature, APEX_IMS, APEX_matrix, targetP signal, mito protein domain, MS/MS++MIMMetabolism > Carbohydrate metabolism > Malate-aspartate shuttle;
Metabolism > Amino acid metabolism > Glutamate metabolism;
Metabolism > Amino acid metabolism > Glyoxylate metabolism;
Metabolism > Amino acid metabolism > Kynurenine metabolism;
Metabolism > Sulfur metabolism
all 14
NAXDNAD(P)HX ­dehydrataseCARKD, LP3298, PEBEL2, Q8IW45, gene:557398GFP, targetP signal, mito protein domain, MS/MS++MIMMetabolism > Metals and cofactors > NAD biosynthesis and metabolismall 14
MT-ND3NADH ­dehydrogenase, ­subunit ­3 ­(complex ­I)MTND3, P03897, gene:45378literature, Rickettsial homolog, mito protein domain+, MS/MSMIMOXPHOS > Complex I > CI subunits;
OXPHOS > OXPHOS subunits
cerebrum, cerebellum, brainstem, spinalcord, kidney, heart, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta
ACCS1-aminocyclopropane-1-carboxylate ­synthase ­homolog ­(inactive)ACS, PHACS, Q96QU6, gene:846808yeast mito homolog++, Rickettsial homolog, mito protein domain, coexpressionunknownnonenone
PTRH2peptidyl-tRNA ­hydrolase ­2BIT1, CFAP37, CGI-147, IMNEPD, PTH, PTH 2, PTH2, Q9Y3E5, gene:516518literature, yeast mito homolog, mito protein domain, MS/MS++MOMnoneall 14
PGS1phosphatidylglycerophosphate ­synthase ­1Q32NB8, gene:94898targetP signal, yeast mito homolog++, mito protein domainMIMMetabolism > Lipid metabolism > Cardiolipin synthesis;
Metabolism > Lipid metabolism > Phospholipid metabolism
cerebrum, spinalcord, placenta
OXR1oxidation ­resistance ­1CHEGDD, Nbla00307, TLDC3, Q8N573, gene:550748literature, yeast mito homolog+, mito protein domain, MS/MSMOMMetabolism > Detoxification > ROS and glutathione metabolismcerebrum, cerebellum, brainstem
LYPLAL1lysophospholipase ­like ­1Q96AV0, Q5VWZ2, gene:1270188mito protein domain, MS/MS++unknownMetabolism > Lipid metabolismcerebrum, cerebellum, brainstem, spinalcord, kidney, liver, adipose, largeintestine, placenta, testis
DNAJC28DnaJ ­heat ­shock ­protein ­family ­(Hsp40) ­member ­C28C21orf55, C21orf78, Q9NX36, gene:549438Rickettsial homolog, mito protein domain, coexpression+unknownnonestomach
FPGSfolylpolyglutamate ­synthaseQ05932, gene:23568APEX_matrix, GFP, targetP signal, yeast mito homolog, Rickettsial homolog, mito protein domainMatrixMetabolism > Vitamin metabolism > Folate and 1-C metabolismnone
TMEM205transmembrane ­protein ­205UNQ501, Q6UW68, gene:3748828yeast mito homolog, mito protein domain+, coexpressionMembranenoneliver, adipose
SLC25A53solute ­carrier ­family ­25 ­member ­53MCART6, Q5H9E4, gene:4016128yeast mito homolog+, mito protein domain+MIMSmall molecule transport > SLC25A familytestis
AIFM3apoptosis ­inducing ­factor ­mitochondria ­associated ­3AIFL, Q96NN9, gene:1502098literature, yeast mito homolog, Rickettsial homolog, mito protein domain+MIMMetabolism > Metals and cofactors > Fe-S-containing proteins;
Mitochondrial dynamics and surveillance > Apoptosis
none
MGST1microsomal ­glutathione ­S-transferase ­1GST12, MGST, MGST-I, P10620, gene:42578mito protein domain, coexpression, MS/MS++MembraneMetabolism > Detoxification > Xenobiotic metabolism;
Metabolism > Detoxification > ROS and glutathione metabolism
cerebrum, brainstem, spinalcord, liver, heart, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
MTFP1mitochondrial ­fission ­process ­1HSPC242, MTP18, Q9UDX5, gene:515378literature, induction, coexpression, MS/MS+MembraneMitochondrial dynamics and surveillance > Fissionall 14
COA4cytochrome ­c ­oxidase ­assembly ­factor ­4 ­homologCHCHD8, CMC3, E2IG2, Q9NYJ1, gene:512878yeast mito homolog++, mito protein domain, MS/MSIMSOXPHOS > Complex IV > CIV assembly factors;
OXPHOS > OXPHOS assembly factors
placenta
NIF3L1NGG1 ­interacting ­factor ­3 ­like ­1ALS2CR1, CALS-7, MDS015, Q9GZT8, gene:604918APEX_matrix, targetP signal, yeast mito homolog, coexpression, MS/MS+Matrixnonecerebrum, cerebellum, brainstem, spinalcord, skeletalmuscle, adipose, largeintestine, placenta, testis
PUSL1pseudouridine ­synthase ­like ­1Q8N0Z8, gene:1267898APEX_matrix, yeast mito homolog+, Rickettsial homolog, mito protein domainMatrixMitochondrial central dogma > Translationcerebrum, spinalcord, largeintestine, placenta
SDSLserine ­dehydratase ­likeSDH 2, SDS-RS1, TDH, cSDH, Q96GA7, gene:1136758yeast mito homolog+, Rickettsial homolog, mito protein domain, coexpressionunknownMetabolism > Amino acid metabolism > Serine metabolismnone
BAXBCL2 ­associated ­X, ­apoptosis ­regulatorBCL2L4, Q07812, gene:5818literature, mito protein domain, coexpression, MS/MS++MOMMitochondrial dynamics and surveillance > Apoptosiscerebrum, cerebellum, brainstem, adipose, largeintestine, placenta, testis
MAVSmitochondrial ­antiviral ­signaling ­proteinCARDIF, IPS-1, IPS1, VISA, Q7Z434, gene:575068literature, induction, coexpression+, MS/MSMOMSignaling > Immune responsebrainstem, skeletalmuscle, adipose, placenta, testis
METTL17methyltransferase ­like ­17METT11D1, Q9H7H0, gene:647458GFP, yeast mito homolog++, mito protein domain+MatrixMitochondrial central dogma > mtRNA metabolism > mt-rRNA modifications;
Mitochondrial central dogma > Translation > Mitochondrial ribosome assembly
none
ACACBacetyl-CoA ­carboxylase ­betaACC2, ACCB, HACC275, O00763, gene:328Rickettsial homolog, mito protein domain, coexpression+MOMMetabolism > Lipid metabolism > Fatty acid oxidation;
Metabolism > Vitamin metabolism > Biotin utilizing proteins
cerebrum, cerebellum, brainstem, spinalcord, kidney, liver, adipose, largeintestine, stomach, placenta, testis
ARG2arginase ­2P78540, gene:3848literature, APEX_matrix, targetP signal+, mito protein domain, MS/MSMatrixMetabolism > Amino acid metabolismkidney, adipose, smallintestine, largeintestine, placenta
MTPAPmitochondrial ­poly(A) ­polymerasePAPD1, SPAX4, TENT6, Q9NVV4, gene:551497literature, APEX_matrix, targetP signal+, mito protein domain, inductionMatrixMitochondrial central dogma > mtRNA metabolism > mtRNA granules;
Mitochondrial central dogma > mtRNA metabolism > mt-rRNA modifications;
Mitochondrial central dogma > mtRNA metabolism > mt-mRNA modifications;
Mitochondrial central dogma > mtRNA metabolism > mtRNA stability and decay
cerebrum, cerebellum, spinalcord, liver, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
CCDC51coiled-coil ­domain ­containing ­51MITOK, Q96ER9, gene:797147APEX_matrix, targetP signal, MS/MS++MIMSmall molecule transportall 14
SUGCTsuccinyl-CoA:glutarate-CoA ­transferaseC7orf10, DERP13, GA3, ORF19, Q9HAC7, gene:797837APEX_matrix, targetP signal, mito protein domain, coexpression, MS/MS+MatrixMetabolism > Amino acid metabolismkidney, liver, testis
DNM1Ldynamin ­1 ­likeDLP1, DRP1, DVLP, DYMPLE, EMPF, EMPF1, HDYNIV, OPA5, O00429, gene:100597literature, yeast mito homolog+, mito protein domain, inductionMOMMitochondrial dynamics and surveillance > Fissioncerebrum, cerebellum, brainstem, spinalcord
NRDCnardilysin ­convertaseNRD1, hNRD1, hNRD2, O43847, gene:48987targetP signal, Rickettsial homolog, mito protein domain+MatrixProtein import, sorting and homeostasis > Protein homeostasis > Chaperones;
Protein import, sorting and homeostasis > Protein import and sorting
testis
TMEM126Btransmembrane ­protein ­126BHT007, MC1DN29, Q8IUX1, gene:558637targetP signal, mito protein domain+, MS/MSMIMOXPHOS > Complex I > CI assembly factors;
OXPHOS > OXPHOS assembly factors
cerebrum, cerebellum, brainstem, spinalcord, liver, heart, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
NSUN4NOP2/Sun ­RNA ­methyltransferase ­4SHTAP, Q96CB9, gene:3873387APEX_matrix, targetP signal, mito protein domain, induction, coexpressionMatrixMitochondrial central dogma > mtRNA metabolism > mt-rRNA modifications;
Mitochondrial central dogma > Translation > Mitochondrial ribosome assembly
placenta
ACSS3acyl-CoA ­synthetase ­short ­chain ­family ­member ­3Q9H6R3, gene:796117Rickettsial homolog, mito protein domain, MS/MSMatrixMetabolism > Lipid metabolismkidney, liver, adipose, placenta, testis
FAM210Bfamily ­with ­sequence ­similarity ­210 ­member ­B5A3, C20orf108, dJ1167H4.1, Q96KR6, gene:1161517targetP signal, yeast mito homolog, coexpression, MS/MSMIMMetabolism > Metals and cofactors > Iron homeostasiscerebrum, cerebellum, brainstem, spinalcord, liver, adipose, smallintestine, largeintestine, stomach, placenta, testis
RBFAribosome ­binding ­factor ­AC18orf22, HsT169, Q8N0V3, gene:798637APEX_matrix, targetP signal+, Rickettsial homolog, coexpressionMatrixMitochondrial central dogma > Translation > Mitochondrial ribosome assemblynone
METAP1Dmethionyl ­aminopeptidase ­type ­1D, ­mitochondrialMAP 1D, MAP1D, MetAP 1D, Metap1l, Q6UB28, gene:2540427literature, APEX_matrix, targetP signal, Rickettsial homolog, mito protein domain, coexpressionMatrixMitochondrial central dogma > Translation > fMet processing;
Protein import, sorting and homeostasis > Protein homeostasis > Proteases
none
AHCYL1adenosylhomocysteinase ­like ­1DCAL, IRBIT, PPP1R78, PRO0233, XPVKONA, O43865, gene:107687targetP signal, mito protein domain+, inductionMOMMitochondrial dynamics and surveillance > Organelle contact sitesbrainstem, spinalcord, smallintestine, stomach
NIPSNAP3Anipsnap ­homolog ­3AHSPC299, NIPSNAP4, TASSC, Q9UFN0, gene:259347APEX_matrix, targetP signal+, MS/MS++Matrixnonecerebrum, cerebellum, brainstem, spinalcord, kidney, liver, adipose, smallintestine, largeintestine, stomach, placenta, testis
MTARC1mitochondrial ­amidoxime ­reducing ­component ­1MARC1, MOSC1, Q5VT66, gene:647577mito protein domain+, MS/MSMOMMetabolism > Metals and cofactors > Molybdenum cofactor synthesis and proteins;
Metabolism > Detoxification > Xenobiotic metabolism;
Metabolism > Detoxification > Amidoxime reducing complex
cerebrum, cerebellum, brainstem, spinalcord, kidney, liver, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
FUNDC1FUN14 ­domain ­containing ­1Q8IVP5, gene:1393417yeast mito homolog, mito protein domain+, MS/MSMOMMitochondrial dynamics and surveillance > Mitophagy;
Mitochondrial dynamics and surveillance > Autophagy
cerebrum, cerebellum, brainstem, spinalcord, kidney, skeletalmuscle, adipose, largeintestine, stomach, testis
ACSL1acyl-CoA ­synthetase ­long ­chain ­family ­member ­1ACS1, FACL1, FACL2, LACS, LACS1, LACS2, P33121, gene:21807Rickettsial homolog, mito protein domain, coexpressionMOMMetabolism > Lipid metabolism > Fatty acid oxidationall 14
COX20cytochrome ­c ­oxidase ­assembly ­factor ­COX20FAM36A, Q5RI15, gene:1162287literature, APEX_IMS, GFP, yeast mito homolog++, MS/MSMIMOXPHOS > Complex IV > CIV assembly factors;
OXPHOS > OXPHOS assembly factors
all 14
HSDL1hydroxysteroid ­dehydrogenase ­like ­1SDR12C3, Q3SXM5, gene:836937GFP, Rickettsial homolog, mito protein domain, MS/MS++unknownnonecerebrum, cerebellum, brainstem, spinalcord, adipose, smallintestine, largeintestine, stomach, placenta, testis
CBR3carbonyl ­reductase ­3HEL-S-25, SDR21C2, hCBR3, O75828, gene:8747targetP signal, Rickettsial homolog, mito protein domain, MS/MSMatrixMetabolism > Detoxification > Xenobiotic metabolismcerebrum, spinalcord, adipose, smallintestine, largeintestine, stomach
MTERF4mitochondrial ­transcription ­termination ­factor ­4MTERFD2, Q7Z6M4, gene:1309167APEX_matrix, targetP signal, mito protein domain+, coexpressionMatrixMitochondrial central dogma > Translation > Mitochondrial ribosome assemblynone
NUDT19nudix ­hydrolase ­19RP2, A8MXV4, gene:3909167APEX_matrix, targetP signal, mito protein domain, inductionMatrixMetabolism > Metals and cofactors > Coenzyme A metabolismkidney, liver, largeintestine, placenta
HEBP1heme ­binding ­protein ­1HBP, HEBP, Q9NRV9, gene:508657APEX_IMS, mito protein domain, coexpression, MS/MS++IMSnonecerebrum, cerebellum, liver, adipose, placenta
PTPMT1protein ­tyrosine ­phosphatase ­mitochondrial ­1DUSP23, MOSP, PLIP, PNAS-129, Q8WUK0, gene:1149717literature, APEX_matrix, mito protein domain, coexpression, MS/MS++MIMMetabolism > Lipid metabolism > Cardiolipin synthesis;
Metabolism > Lipid metabolism > Phospholipid metabolism
all 14
PGAM5PGAM ­family ­member ­5, ­mitochondrial ­serine/threonine ­protein ­phosphataseBXLBV68, Q96HS1, gene:1921117APEX_IMS, mito protein domain, coexpression, MS/MS++MOMMitochondrial dynamics and surveillance > Mitophagy;
Mitochondrial dynamics and surveillance > Autophagy
cerebrum, cerebellum, brainstem, spinalcord, skeletalmuscle, adipose, largeintestine, placenta, testis
DTYMKdeoxythymidylate ­kinaseCDC8, PP3731, TMPK, TYMK, P23919, gene:18417Rickettsial homolog, MS/MS+MatrixMetabolism > Nucleotide metabolism > Nucleotide synthesis and processingcerebrum, cerebellum, brainstem, spinalcord, liver, heart, adipose, smallintestine, largeintestine, stomach, placenta, testis
KARS1lysyl-tRNA ­synthetase ­1CMTRIB, DFNB89, KARS, KARS2, KRS, Q15046, gene:37357literature, GFP, targetP signal, Rickettsial homolog, mito protein domain, coexpressionMatrixMitochondrial central dogma > Translation > mt-tRNA synthetasescerebrum, cerebellum, brainstem, spinalcord, skeletalmuscle, adipose, largeintestine, stomach, placenta, testis
STARsteroidogenic ­acute ­regulatory ­proteinSTARD1, P49675, gene:67707literature, targetP signal, mito protein domain, coexpression, MS/MSMOM, IMSMetabolism > Lipid metabolism > Cholesterol, bile acid, steroid synthesistestis
DNAJC19DnaJ ­heat ­shock ­protein ­family ­(Hsp40) ­member ­C19PAM18, TIM14, TIMM14, Q96DA6, gene:1311187literature, APEX_matrix, mito protein domain, MS/MS++MIMProtein import, sorting and homeostasis > Protein import and sorting > Import motorall 14
OTCornithine ­carbamoyltransferaseOCTD, P00480, gene:50097literature, targetP signal, mito protein domain, MS/MS+MatrixMetabolism > Amino acid metabolism > Urea cyclecerebellum, spinalcord, kidney, liver, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta
COX7A2Lcytochrome ­c ­oxidase ­subunit ­7A2 ­likeCOX7AR, COX7RP, EB1, SIG81, O14548, gene:91677literature, APEX_IMS, APEX_matrix, mito protein domain+, MS/MS+MIMOXPHOS > Complex IV > CIV subunits;
OXPHOS > OXPHOS subunits;
OXPHOS > OXPHOS assembly factors;
OXPHOS > Respirasome assembly
all 14
MTERF3mitochondrial ­transcription ­termination ­factor ­3CGI-12, MTERFD1, Q96E29, gene:510017literature, APEX_matrix, GFP, targetP signal, mito protein domain+, coexpressionMatrixMitochondrial central dogma > Translation > Mitochondrial ribosome assemblynone
SFXN2sideroflexin ­2SLC56A2, Q96NB2, gene:1189807mito protein domain+, coexpressionMIMSmall molecule transport > Sideroflexinskidney, liver, adipose, largeintestine, stomach, placenta, testis
FASNfatty ­acid ­synthaseFAS, OA-519, SDR27X1, P49327, gene:21947Rickettsial homolog, mito protein domain, coexpressionIMSMetabolism > Lipid metabolismcerebrum, cerebellum, brainstem, spinalcord, liver, skeletalmuscle, adipose, smallintestine, stomach, placenta, testis
PINK1PTEN ­induced ­kinase ­1BRPK, PARK6, Q9BXM7, gene:650187literature, targetP signal+, mito protein domain, coexpressionMOMMitochondrial dynamics and surveillance > Mitophagy;
Mitochondrial dynamics and surveillance > Autophagy;
Signaling
none
TMEM177transmembrane ­protein ­177Q53S58, gene:807757targetP signal, coexpression, MS/MSMIMOXPHOS > Complex IV > CIV assembly factors;
OXPHOS > OXPHOS assembly factors
cerebrum, adipose, stomach, placenta, testis
TXNRD1thioredoxin ­reductase ­1GRIM-12, TR, TR1, TRXR1, TXNR, Q16881, gene:72966GFP, Rickettsial homolog, mito protein domain, coexpression, MS/MSunknownMetabolism > Detoxification > ROS and glutathione metabolism;
Metabolism > Detoxification > Selenoproteins
skeletalmuscle, smallintestine, placenta, testis
THNSL1threonine ­synthase ­like ­1TSH1, Q8IYQ7, gene:798966APEX_matrix, mito protein domain, MS/MS++Matrixnonecerebrum, cerebellum, brainstem, spinalcord, skeletalmuscle, adipose, largeintestine, stomach, testis
FDPSfarnesyl ­diphosphate ­synthaseFPPS, FPS, POROK9, P14324, gene:22246targetP signal, mito protein domain, MS/MS+MIMMetabolism > Lipid metabolism > Cholesterol-associatedliver, adipose, smallintestine, largeintestine, placenta
IDI1isopentenyl-diphosphate ­delta ­isomerase ­1IPP1, IPPI1, Q13907, gene:34226targetP signal, mito protein domain, MS/MS+MatrixMetabolism > Lipid metabolism > Cholesterol-associatedcerebrum, cerebellum, brainstem, spinalcord, kidney, liver, adipose, smallintestine, largeintestine, stomach, placenta, testis
TOP3ADNA ­topoisomerase ­III ­alphaMGRISCE2, PEOB5, TOP3, ZGRF7, Q13472, gene:71566APEX_matrix, targetP signal+, Rickettsial homolog, mito protein domainMatrixMitochondrial central dogma > mtDNA maintenance > mtDNA replicationtestis
FTMTferritin ­mitochondrialMTF, Q8N4E7, gene:940336literature, targetP signal+, mito protein domain, MS/MSMatrixMetabolism > Metals and cofactors > Iron homeostasistestis
LDHAL6Blactate ­dehydrogenase ­A ­like ­6BLDH6B, LDHAL6, LDHL, Q9BYZ2, gene:924836targetP signal, Rickettsial homolog, mito protein domain, MS/MSMatrixMetabolism > Carbohydrate metabolismcerebrum, cerebellum, brainstem, spinalcord, kidney, liver, adipose, smallintestine, largeintestine, stomach, placenta, testis
MTFR1mitochondrial ­fission ­regulator ­1CHPPR, FAM54A2, Q15390, gene:96506literature, targetP signal, mito protein domain+, coexpressionMembraneMitochondrial dynamics and surveillance > Fissionnone
BCL2L13BCL2 ­like ­13BCL-RAMBO, Bcl2-L-13, MIL1, Q9BXK5, gene:237866literature, mito protein domain, coexpression, MS/MS+MOMMitochondrial dynamics and surveillance > Mitophagy;
Mitochondrial dynamics and surveillance > Autophagy;
Mitochondrial dynamics and surveillance > Apoptosis
cerebrum, cerebellum, brainstem, spinalcord, kidney, liver, heart, skeletalmuscle, adipose, largeintestine, stomach, placenta, testis
TRMT10CtRNA ­methyltransferase ­10C, ­mitochondrial ­RNase ­P ­subunitCOXPD30, HNYA, MRPP1, RG9MTD1, Q7L0Y3, gene:549316literature, APEX_matrix, GFP, targetP signal+, mito protein domain, coexpressionMatrixMitochondrial central dogma > mtRNA metabolism > mtRNA granules;
Mitochondrial central dogma > mtRNA metabolism > Polycistronic mtRNA processing;
Mitochondrial central dogma > mtRNA metabolism > mt-tRNA modifications
largeintestine, placenta
ACOT7acyl-CoA ­thioesterase ­7ACH1, ACT, BACH, CTE-II, LACH, LACH1, hBACH, O00154, gene:113326targetP signal+, mito protein domain, inductionMatrixMetabolism > Lipid metabolismbrainstem, spinalcord, adipose, largeintestine
TFB1Mtranscription ­factor ­B1, ­mitochondrialCGI-75, CGI75, mtTFB, mtTFB1, Q8WVM0, gene:511066literature, APEX_matrix, Rickettsial homolog, mito protein domain, MS/MS+MatrixMitochondrial central dogma > mtRNA metabolism > mtRNA granules;
Mitochondrial central dogma > mtRNA metabolism > mt-rRNA modifications;
Mitochondrial central dogma > Translation > Mitochondrial ribosome assembly
brainstem, largeintestine, placenta, testis
ARF5ADP ­ribosylation ­factor ­5P84085, gene:3816targetP signal, mito protein domain, coexpression, MS/MSunknownnonecerebrum, cerebellum, brainstem, spinalcord, liver, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
MCUBmitochondrial ­calcium ­uniporter ­dominant ­negative ­subunit ­betaCCDC109B, Q9NWR8, gene:550136literature, targetP signal, mito protein domain+MIMSignaling > Calcium homeostasis > Calcium cycle;
Small molecule transport > Calcium uniporter
placenta
TWNKtwinkle ­mtDNA ­helicaseATXN8, C10orf2, IOSCA, MTDPS7, PEO, PEO1, PEOA3, PRLTS5, SANDO, SCA8, TWINL, Q96RR1, gene:566526literature, APEX_matrix, targetP signal, induction, coexpressionMatrixMitochondrial central dogma > mtDNA maintenance > mtDNA replication;
Mitochondrial central dogma > mtDNA maintenance > mtDNA nucleoid
none
MMADHCmetabolism ­of ­cobalamin ­associated ­DC2orf25, CL25022, cblD, Q9H3L0, gene:272496APEX_matrix, targetP signal, induction, coexpressionMatrixMetabolism > Vitamin metabolism > Vitamin B12 metabolismnone
PYCR2pyrroline-5-carboxylate ­reductase ­2HLD10, P5CR2, Q96C36, gene:299206APEX_matrix, mito protein domain, MS/MS++MatrixMetabolism > Amino acid metabolism > Proline metabolismcerebrum, cerebellum, brainstem, spinalcord, adipose, largeintestine, stomach, placenta, testis
TSTD1thiosulfate ­sulfurtransferase ­like ­domain ­containing ­1KAT, TST, Q8NFU3, gene:1001311876Rickettsial homolog, mito protein domain, MS/MSunknownMetabolism > Sulfur metabolismkidney, smallintestine, largeintestine
LYRM1LYR ­motif ­containing ­1A211C6.1, O43325, gene:571496GFP, mito protein domain+, MS/MSunknownnonebrainstem, testis
OSBPL1Aoxysterol ­binding ­protein ­like ­1AORP-1, ORP1, OSBPL1B, Q9BXW6, gene:1148766Rickettsial homolog, mito protein domain, induction, MS/MSunknownMetabolism > Lipid metabolism > Cholesterol, bile acid, steroid synthesis;
Metabolism > Lipid metabolism > Phospholipid metabolism
cerebrum, liver, largeintestine, placenta
RECQL4RecQ ­like ­helicase ­4RECQ4, O94761, gene:94016targetP signal, Rickettsial homolog, mito protein domain, coexpressionMatrixMitochondrial central dogma > mtDNA maintenance > mtDNA repairnone
PET100PET100 ­cytochrome ­c ­oxidase ­chaperoneC19orf79, P0DJ07, gene:1001318016literature, yeast mito homolog++, MS/MSMIMOXPHOS > Complex IV > CIV assembly factors;
OXPHOS > OXPHOS assembly factors
cerebrum, spinalcord, kidney, liver, heart, smallintestine, largeintestine, placenta
GDAP1ganglioside ­induced ­differentiation ­associated ­protein ­1CMT4, CMT4A, CMTRIA, Q8TB36, gene:543326APEX_IMS, mito protein domain, MS/MS++MOMnonecerebrum, cerebellum, brainstem, spinalcord
MTIF3mitochondrial ­translational ­initiation ­factor ­3IF3mt, Q9H2K0, gene:2194026literature, APEX_matrix, Rickettsial homolog, mito protein domain, coexpressionMatrixMitochondrial central dogma > Translation > Translation factorslargeintestine
UNGuracil ­DNA ­glycosylaseDGU, HIGM4, HIGM5, UDG, UNG1, UNG15, UNG2, P13051, gene:73746literature, targetP signal, yeast mito homolog, mito protein domain, inductionMatrixMitochondrial central dogma > mtDNA maintenance > mtDNA repairnone
GLYATglycine-N-acyltransferaseACGNAT, CAT, GAT, Q6IB77, gene:102496literature, mito protein domain, coexpression, MS/MS+MatrixMetabolism > Amino acid metabolism > Glycine metabolism;
Metabolism > Detoxification > Xenobiotic metabolism
spinalcord, kidney, liver, placenta
ISOC2isochorismatase ­domain ­containing ­2Q96AB3, gene:797636APEX_matrix, targetP signal, MS/MS++Matrixnoneall 14
ATP5MC1ATP ­synthase ­membrane ­subunit ­c ­locus ­1ATP5A, ATP5G, ATP5G1, P05496, gene:5166literature, yeast mito homolog+, Rickettsial homolog, mito protein domainMIMOXPHOS > Complex V > CV subunits;
OXPHOS > OXPHOS subunits
none
QDPRquinoid ­dihydropteridine ­reductaseDHPR, HDHPR, PKU2, SDR33C1, P09417, gene:58606APEX_matrix, targetP signal, mito protein domain, inductionMatrixMetabolism > Metals and cofactors > Tetrahydobiopterin synthesis;
Metabolism > Vitamin metabolism > Folate and 1-C metabolism
cerebrum, cerebellum, brainstem, spinalcord, kidney, liver, adipose, stomach, placenta, testis
COQ8Bcoenzyme ­Q8BADCK4, NPHS9, Q96D53, gene:799346APEX_matrix, GFP, yeast mito homolog+, Rickettsial homolog, mito protein domainMIMMetabolism > Metals and cofactors > Coenzyme Q metabolismadipose, smallintestine, largeintestine, placenta
GTPBP10GTP ­binding ­protein ­10ObgH2, UG0751c10, A4D1E9, gene:858656APEX_matrix, yeast mito homolog+, Rickettsial homolog, mito protein domainMatrixMitochondrial central dogma > Translation > Mitochondrial ribosome assemblylargeintestine, placenta
TSPOtranslocator ­proteinBPBS, BZRP, DBI, IBP, MBR, PBR, PBS, PKBS, PTBR, mDRC, pk18, P30536, gene:7066literature, mito protein domain, MS/MS++MOMMetabolism > Lipid metabolism > Cholesterol, bile acid, steroid synthesisspinalcord, kidney, liver, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
FTH1ferritin ­heavy ­chain ­1FHC, FTH, FTHL6, HFE5, PIG15, PLIF, P02794, gene:24956mito protein domain, MS/MS++unknownMetabolism > Metals and cofactors > Iron homeostasiscerebrum, cerebellum, brainstem, spinalcord, liver, adipose, smallintestine, largeintestine, stomach, placenta
TRMT1tRNA ­methyltransferase ­1MRT68, TRM1, Q9NXH9, gene:556216targetP signal+, mito protein domain, coexpressionMatrixMitochondrial central dogma > mtRNA metabolism > mt-tRNA modificationsnone
ADCK2aarF ­domain ­containing ­kinase ­2AARF, Q7Z695, gene:909566GFP, targetP signal, yeast mito homolog, Rickettsial homolog, mito protein domainMIMnonenone
TSTD3thiosulfate ­sulfurtransferase ­like ­domain ­containing ­3H0UI37, gene:1001308906mito protein domain, induction, MS/MSunknownnonecerebrum, cerebellum, brainstem, spinalcord, kidney, liver, adipose, smallintestine, largeintestine, stomach, placenta, testis
MRPS36mitochondrial ­ribosomal ­protein ­S36DC47, MRP-S36, P82909, gene:922596literature, APEX_matrix, MS/MS++MatrixMitochondrial central dogma > Translation > Mitochondrial ribosome;
Metabolism > Carbohydrate metabolism > TCA cycle
all 14
PDP2pyruvate ­dehyrogenase ­phosphatase ­catalytic ­subunit ­2PDPC 2, PPM2B, PPM2C2, Q9P2J9, gene:575466APEX_matrix, yeast mito homolog+, mito protein domain, coexpressionMatrixMetabolism > Carbohydrate metabolism > Pyruvate metabolismadipose
AGPAT51-acylglycerol-3-phosphate ­O-acyltransferase ­51AGPAT5, LPAATE, Q9NUQ2, gene:553266literature, Rickettsial homolog, mito protein domain, induction, MS/MSMOMMetabolism > Lipid metabolism > Cardiolipin synthesis;
Metabolism > Lipid metabolism > Phospholipid metabolism
cerebellum, brainstem, spinalcord, adipose, smallintestine, largeintestine, placenta, testis
MCRIP2MAPK ­regulated ­corepressor ­interacting ­protein ­2C16orf14, FAM195A, c349E10.1, Q9BUT9, gene:843315induction, coexpression+unknownSignalingnone
UCP2uncoupling ­protein ­2BMIQ4, SLC25A8, UCPH, P55851, gene:73515literature, yeast mito homolog+, mito protein domain+MIMSmall molecule transport > SLC25A familynone
SLC25A37solute ­carrier ­family ­25 ­member ­37HT015, MFRN, MFRN1, MSC, MSCP, PRO1278, PRO1584, PRO2217, Q9NYZ2, gene:513125literature, yeast mito homolog+, mito protein domain+MIMMetabolism > Metals and cofactors > Iron homeostasis;
Small molecule transport > SLC25A family
none
SLC25A28solute ­carrier ­family ­25 ­member ­28MFRN2, MRS3/4, MRS4L, NPD016, Q96A46, gene:818945literature, yeast mito homolog+, mito protein domain+MIMMetabolism > Metals and cofactors > Iron homeostasis;
Small molecule transport > SLC25A family
none
SLC25A48solute ­carrier ­family ­25 ­member ­48Q6ZT89, gene:1533285yeast mito homolog+, mito protein domain+MIMSmall molecule transport > SLC25A familynone
SLC25A43solute ­carrier ­family ­25 ­member ­43Q8WUT9, gene:2034275yeast mito homolog+, mito protein domain+MIMSmall molecule transport > SLC25A familynone
SLC25A47solute ­carrier ­family ­25 ­member ­47C14orf68, HDMCP, HMFN1655, Q6Q0C1, gene:2836005literature, yeast mito homolog+, mito protein domain+MIMSmall molecule transport > SLC25A familynone
SLC25A41solute ­carrier ­family ­25 ­member ­41APC4, Q8N5S1, gene:2844275yeast mito homolog+, mito protein domain+MIMMetabolism > Nucleotide metabolism > Nucleotide import;
Small molecule transport > SLC25A family
none
SLC25A34solute ­carrier ­family ­25 ­member ­34Q6PIV7, gene:2847235yeast mito homolog+, mito protein domain+MIMSmall molecule transport > SLC25A familynone
EPHX2epoxide ­hydrolase ­2ABHD20, CEH, SEH, P34913, gene:20535Rickettsial homolog, mito protein domain, coexpressionunknownMetabolism > Detoxification > Xenobiotic metabolism;
Metabolism > Lipid metabolism
kidney, liver, heart, skeletalmuscle, adipose, smallintestine, stomach
NUDT5nudix ­hydrolase ­5YSA1, YSA1H, YSAH1, hNUDT5, Q9UKK9, gene:111645yeast mito homolog++, mito protein domain, coexpressionunknownMetabolism > Nucleotide metabolism > Nucleotide synthesis and processingnone
MICU1mitochondrial ­calcium ­uptake ­1CALC, CBARA1, EFHA3, MPXPS, ara CALC, Q9BPX6, gene:103675literature, APEX_IMS, GFP, targetP signal, mito protein domain, MS/MS+MIMSignaling > Calcium homeostasis > Calcium cycle;
Signaling > Calcium homeostasis > EF hand proteins;
Small molecule transport > Calcium uniporter
cerebrum, cerebellum, brainstem, spinalcord, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
SOD1superoxide ­dismutase ­1ALS, ALS1, HEL-S-44, IPOA, SOD, STAHP, hSod1, homodimer, P00441, gene:66475literature, mito protein domain, induction, coexpressionIMSMetabolism > Detoxification > ROS and glutathione metabolismall 14
SERHL2serine ­hydrolase ­like ­2dJ222E13.1, Q9H4I8, gene:2531905Rickettsial homolog, mito protein domain, MS/MSunknownnonekidney, adipose, smallintestine, largeintestine, stomach, placenta, testis
NDUFAF3NADH:ubiquinone ­oxidoreductase ­complex ­assembly ­factor ­32P1, C3orf60, E3-3, MC1DN18, Q9BU61, gene:259155literature, targetP signal, mito protein domain, MS/MSMIMOXPHOS > Complex I > CI assembly factors;
OXPHOS > OXPHOS assembly factors
all 14
BOLA3bolA ­family ­member ­3MMDS2, Q53S33, gene:3889625targetP signal, mito protein domain, MS/MSMatrixMetabolism > Metals and cofactors > Fe-S cluster biosynthesis;
Metabolism > Metals and cofactors > Fe-S-containing proteins
cerebrum, spinalcord, liver, heart, adipose, smallintestine, largeintestine, stomach, placenta, testis
OCIAD2OCIA ­domain ­containing ­2Q56VL3, gene:1322995MS/MS++unknownSignalingall 14
RSAD1radical ­S-adenosyl ­methionine ­domain ­containing ­1HemW, Q9HA92, gene:553165APEX_matrix, targetP signal, Rickettsial homolog, mito protein domain, coexpressionMIMMetabolism > Metals and cofactors > Fe-S-containing proteinsnone
CHCHD6coiled-coil-helix-coiled-coil-helix ­domain ­containing ­6CHCM1, MICOS25, Mic25, PPP1R23, Q9BRQ6, gene:843035literature, APEX_IMS, APEX_matrix, coexpression, MS/MS++MIMMitochondrial dynamics and surveillance > Cristae formation > MICOS complexcerebrum, cerebellum, brainstem, spinalcord, kidney, liver, heart, adipose, smallintestine, largeintestine, stomach, placenta, testis
FASTKD2FAST ­kinase ­domains ­2COXPD44, KIAA0971, Q9NYY8, gene:228685literature, APEX_matrix, induction, coexpression, MS/MSMatrixMitochondrial central dogma > mtRNA metabolism > mtRNA granules;
Mitochondrial central dogma > mtRNA metabolism > Polycistronic mtRNA processing;
Mitochondrial central dogma > mtRNA metabolism > mtRNA stability and decay;
Mitochondrial central dogma > Translation > Mitochondrial ribosome assembly
skeletalmuscle, adipose, smallintestine, largeintestine, placenta, testis
PEX11Bperoxisomal ­biogenesis ­factor ­11 ­betaPEX11-BETA, PEX14B, O96011, gene:87995targetP signal, coexpression, MS/MSMembranenonecerebrum, cerebellum, brainstem, spinalcord, kidney, liver, adipose, smallintestine, largeintestine, placenta, testis
TMEM186transmembrane ­protein ­186C16orf51, Q96B77, gene:258805targetP signal, coexpression, MS/MSMIMOXPHOS > Complex I > CI assembly factors;
OXPHOS > OXPHOS assembly factors
cerebrum, cerebellum, brainstem, spinalcord, liver, heart, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
PIF1PIF1 ­5'-to-3' ­DNA ­helicaseC15orf20, PIF, Q9H611, gene:801195literature, targetP signal+, yeast mito homolog, mito protein domainMatrixMitochondrial central dogma > mtDNA maintenancenone
FABP1fatty ­acid ­binding ­protein ­1FABPL, L-FABP, P07148, gene:21685mito protein domain, MS/MS++unknownMetabolism > Metals and cofactors > Heme synthesis and processing;
Metabolism > Lipid metabolism
liver, smallintestine
ABCD1ATP ­binding ­cassette ­subfamily ­D ­member ­1ABC42, ALD, ALDP, AMN, P33897, gene:2155Rickettsial homolog, mito protein domain, inductionMembraneSmall molecule transport > ABC transportersadipose
PLPBPpyridoxal ­phosphate ­binding ­proteinEPVB6D, PROSC, O94903, gene:112125APEX_matrix, coexpression, MS/MS++MatrixMetabolism > Vitamin metabolism > Vitamin B6 metabolismall 14
DBIdiazepam ­binding ­inhibitor, ­acyl-CoA ­binding ­proteinACBD1, ACBP, CCK-RP, EP, P07108, gene:16225mito protein domain, induction, coexpressionunknownMetabolism > Lipid metabolismcerebrum, cerebellum, brainstem, spinalcord, kidney, liver, adipose, smallintestine, largeintestine, stomach, placenta, testis
PNPT1polyribonucleotide ­nucleotidyltransferase ­1COXPD13, DFNB70, OLD35, PNPASE, old-35, Q8TCS8, gene:871785literature, APEX_matrix, targetP signal, Rickettsial homolog, mito protein domainMatrix, IMSMitochondrial central dogma > mtRNA metabolism > mtRNA stability and decayall 14
TOP1MTDNA ­topoisomerase ­I ­mitochondrialQ969P6, gene:1164475literature, APEX_matrix, targetP signal, mito protein domain, coexpressionMatrixMitochondrial central dogma > mtDNA maintenance > mtDNA replication;
Mitochondrial central dogma > mtRNA metabolism > Transcription
none
RNASEH1ribonuclease ­H1H1RNA, PEOB2, RNH1, O60930, gene:2462435literature, APEX_matrix, targetP signal, Rickettsial homolog, coexpressionMatrixMitochondrial central dogma > mtDNA maintenance > mtDNA replication;
Mitochondrial central dogma > mtRNA metabolism > mtRNA stability and decay
none
ABCA9ATP ­binding ­cassette ­subfamily ­A ­member ­9EST640918, Q8IUA7, gene:103505GFP, yeast mito homolog, Rickettsial homolog, mito protein domainMembraneSmall molecule transport > ABC transporterscerebrum, placenta
SCP2sterol ­carrier ­protein ­2NLTP, NSL-TP, SCP-2, SCP-CHI, SCP-X, SCPX, P22307, gene:63425Rickettsial homolog, mito protein domain+MIMMetabolism > Lipid metabolism > Cholesterol, bile acid, steroid synthesisbrainstem, spinalcord, kidney, liver, heart, adipose, smallintestine, placenta, testis
NT5DC35'-nucleotidase ­domain ­containing ­3TU12B1-TY, TU12B1TY, Q86UY8, gene:515595APEX_matrix, targetP signal, MS/MS++Matrixnonecerebrum, cerebellum, brainstem, spinalcord, skeletalmuscle, stomach, placenta, testis
CMC4C-X9-C ­motif ­containing ­4C6.1B, MTCP1, MTCP1B, MTCP1NB, p8, p8MTCP1, P56277, gene:1002721475yeast mito homolog++, mito protein domain+IMSnonenone
LDHBlactate ­dehydrogenase ­BHEL-S-281, LDH-B, LDH-H, LDHBD, TRG-5, P07195, gene:39455Rickettsial homolog, mito protein domain, inductionunknownMetabolism > Amino acid metabolism > Glyoxylate metabolismcerebrum, cerebellum, brainstem, spinalcord, kidney, liver, adipose, smallintestine, largeintestine, stomach, placenta, testis
YBEYybeY ­metalloendoribonucleaseC21orf57, P58557, gene:540595APEX_matrix, targetP signal, Rickettsial homolog, MS/MSMatrixMitochondrial central dogma > mtRNA metabolism > Polycistronic mtRNA processing;
Mitochondrial central dogma > Translation > Mitochondrial ribosome assembly
largeintestine, placenta, testis
HINT1histidine ­triad ­nucleotide ­binding ­protein ­1HINT, NMAN, PKCI-1, PRKCNH1, P49773, gene:30945Rickettsial homolog, mito protein domain, coexpressionunknownMetabolism > Nucleotide metabolism > Nucleotide synthesis and processingcerebrum, cerebellum, brainstem, spinalcord, kidney, smallintestine, placenta, testis
MUL1mitochondrial ­E3 ­ubiquitin ­protein ­ligase ­1C1orf166, GIDE, MAPL, MULAN, RNF218, Q969V5, gene:795945literature, mito protein domain+, inductionMOMProtein import, sorting and homeostasis > Protein homeostasis;
Mitochondrial dynamics and surveillance > Fission;
Signaling
skeletalmuscle, largeintestine
TK2thymidine ­kinase ­2MTDPS2, MTTK, PEOB3, SCA31, O00142, gene:70845literature, targetP signal, mito protein domain, MS/MSMatrixMetabolism > Nucleotide metabolism > Nucleotide synthesis and processingcerebrum, cerebellum, brainstem, adipose
AGMATagmatinaseQ9BSE5, gene:798145literature, targetP signal, mito protein domain, MS/MS+MatrixMetabolism > Amino acid metabolismliver, smallintestine
CMPK2cytidine/uridine ­monophosphate ­kinase ­2NDK, TMPK2, TYKi, UMP-CMPK2, Q5EBM0, gene:1296074literature, targetP signal+, MS/MSMatrixMetabolism > Nucleotide metabolism > Nucleotide synthesis and processingspinalcord, smallintestine
BIDBH3 ­interacting ­domain ­death ­agonistFP497, P55957, gene:6374literature, targetP signal+, mito protein domain, coexpressionMOMMitochondrial dynamics and surveillance > Apoptosisnone
ARL2ADP ­ribosylation ­factor ­like ­GTPase ­2ARFL2, P36404, gene:4024literature, mito protein domain, coexpression, MS/MS+IMSMitochondrial dynamics and surveillance > Fusioncerebrum, brainstem, spinalcord, adipose, largeintestine
SYNJ2BPsynaptojanin ­2 ­binding ­proteinARIP2, OMP25, P57105, gene:553334APEX_IMS, mito protein domain, coexpression, MS/MS+MOMMitochondrial dynamics and surveillance > Organelle contact sitescerebrum, cerebellum, brainstem, spinalcord, kidney, liver, heart, skeletalmuscle, adipose, largeintestine, stomach, placenta, testis
MFFmitochondrial ­fission ­factorC2orf33, EMPF2, GL004, Q9GZY8, gene:569474literature, induction, MS/MSMOMMitochondrial dynamics and surveillance > Fissioncerebrum, cerebellum, brainstem, spinalcord, adipose, largeintestine, placenta, testis
DCXRdicarbonyl ­and ­L-xylulose ­reductaseDCR, HCR2, HCRII, KIDCR, P34H, PNTSU, SDR20C1, XR, Q7Z4W1, gene:511814APEX_matrix, targetP signal, Rickettsial homolog, mito protein domainMatrixMetabolism > Carbohydrate metabolismcerebrum, cerebellum, spinalcord, heart, adipose, largeintestine, stomach, placenta, testis
NUDT9nudix ­hydrolase ­9NUDT10, Q9BW91, gene:533434APEX_matrix, mito protein domain, coexpression, MS/MSMatrixMetabolism > Nucleotide metabolism > Nucleotide synthesis and processingcerebrum, cerebellum, placenta, testis
AMACRalpha-methylacyl-CoA ­racemaseAMACRD, CBAS4, P504S, RACE, RM, Q9UHK6, gene:236004literature, mito protein domain, coexpression, MS/MSMatrixMetabolism > Lipid metabolism > Fatty acid oxidationkidney, liver, adipose, smallintestine, testis
VPS13Dvacuolar ­protein ­sorting ­13 ­homolog ­DSCAR4, Q5THJ4, gene:551874manual, mito protein domain, induction, coexpressionMOMMitochondrial dynamics and surveillance > Mitophagy;
Mitochondrial dynamics and surveillance > Autophagy
cerebrum, cerebellum, brainstem, spinalcord, kidney, skeletalmuscle
NUDT6nudix ­hydrolase ­6ASFGF2, FGF-AS, FGF2AS, GFG-1, GFG1, P53370, gene:111624APEX_matrix, targetP signal, mito protein domain, coexpressionMatrixnonenone
DMAC2distal ­membrane ­arm ­assembly ­complex ­2ATP5SL, Q9NW81, gene:551014APEX_matrix, GFP, targetP signal, induction, MS/MSMIMOXPHOS > Complex I > CI assembly factors;
OXPHOS > OXPHOS assembly factors
cerebrum, cerebellum, brainstem, stomach
MRM2mitochondrial ­rRNA ­methyltransferase ­2FJH1, FTSJ2, HEL97, MTDPS17, RRMJ2, Q9UI43, gene:299604literature, Rickettsial homolog, mito protein domain, coexpressionMatrixMitochondrial central dogma > mtRNA metabolism > mtRNA granules;
Mitochondrial central dogma > mtRNA metabolism > mt-rRNA modifications;
Mitochondrial central dogma > Translation > Mitochondrial ribosome assembly
cerebrum
BAK1BCL2 ­antagonist/killer ­1BAK, BAK-LIKE, BCL2L7, CDN1, Q16611, gene:5784literature, mito protein domain, MS/MS++MOMMitochondrial dynamics and surveillance > Apoptosisbrainstem, spinalcord, liver, heart, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
DHODHdihydroorotate ­dehydrogenase ­(quinone)DHOdehase, POADS, URA1, Q02127, gene:17234literature, mito protein domain, MS/MS++MIMMetabolism > Nucleotide metabolism > Nucleotide synthesis and processing;
Metabolism > Electron carriers > Q-linked reactions, other
cerebrum, spinalcord, kidney, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
MAOBmonoamine ­oxidase ­BP27338, gene:41294literature, mito protein domain, MS/MS++MOMMetabolism > Amino acid metabolism > Catechol metabolism;
Metabolism > Detoxification > Xenobiotic metabolism
cerebrum, cerebellum, brainstem, spinalcord, liver, heart, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
MGST3microsomal ­glutathione ­S-transferase ­3GST-III, O14880, gene:42594APEX_IMS, mito protein domain, MS/MS++MOMMetabolism > Lipid metabolism > Eicosanoid metabolismall 14
ACP6acid ­phosphatase ­6, ­lysophosphatidicACPL1, LPAP, PACPL1, Q9NPH0, gene:512054literature, APEX_IMS, mito protein domain, MS/MS++IMSMetabolism > Lipid metabolism > Phospholipid metabolismcerebrum, spinalcord, kidney, liver, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
MRPL48mitochondrial ­ribosomal ­protein ­L48CGI-118, HSPC290, L48MT, MRP-L48, Q96GC5, gene:516424literature, APEX_matrix, mito protein domain, MS/MS++MatrixMitochondrial central dogma > Translation > Mitochondrial ribosomeall 14
AGXTalanine--glyoxylate ­and ­serine--pyruvate ­aminotransferaseAGT, AGT1, AGXT1, PH1, SPAT, SPT, TLH6, P21549, gene:1894literature, yeast mito homolog, mito protein domain, MS/MSMatrixMetabolism > Amino acid metabolism > Serine metabolism;
Metabolism > Amino acid metabolism > Glyoxylate metabolism
liver
CYB5R3cytochrome ­b5 ­reductase ­3B5R, DIA1, P00387, gene:17274literature, mito protein domain, MS/MS+MOMMetabolism > Detoxification > Xenobiotic metabolism;
Metabolism > Detoxification > Amidoxime reducing complex;
Metabolism > Lipid metabolism
cerebrum, cerebellum, brainstem, spinalcord, kidney, liver, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
DDX28DEAD-box ­helicase ­28MDDX28, Q9NUL7, gene:557944literature, APEX_matrix, targetP signal, Rickettsial homolog, mito protein domainMatrixMitochondrial central dogma > mtRNA metabolism > mtRNA granules;
Mitochondrial central dogma > Translation > Mitochondrial ribosome assembly
spinalcord, adipose
PNKDPNKD ­metallo-beta-lactamase ­domain ­containingBRP17, DYT8, FKSG19, FPD1, KIPP1184, MR-1, MR-1S, MR1, PDC, PKND1, PNKD1, R1, TAHCCP2, Q8N490, gene:259534literature, targetP signal, mito protein domain, MS/MSMIMOXPHOS > Complex IV > CIV assembly factors;
OXPHOS > OXPHOS assembly factors
cerebrum, cerebellum, spinalcord, liver, adipose, smallintestine, largeintestine, stomach, placenta, testis
MICU3mitochondrial ­calcium ­uptake ­family ­member ­3EFHA2, Q86XE3, gene:2860974manual, targetP signal, mito protein domain, MS/MSMIMSignaling > Calcium homeostasis > Calcium cycle;
Signaling > Calcium homeostasis > EF hand proteins;
Small molecule transport > Calcium uniporter
cerebrum, cerebellum, brainstem, spinalcord, skeletalmuscle, stomach
BNIP3BCL2 ­interacting ­protein ­3NIP3, Q12983, gene:6644literature, mito protein domain+, MS/MSMOMMitochondrial dynamics and surveillance > Apoptosiscerebrum, cerebellum, brainstem, heart, skeletalmuscle, adipose
MTX3metaxin ­3Q5HYI7, gene:3457784manual, mito protein domain+, MS/MSMOMMitochondrial dynamics and surveillance > Intramitochondrial membrane interactionscerebrum, cerebellum, brainstem, spinalcord
MUTYHmutY ­DNA ­glycosylaseMYH, Q9UIF7, gene:45954GFP, targetP signal+, Rickettsial homolog, mito protein domainMatrixMitochondrial central dogma > mtDNA maintenancenone
GTPBP6GTP ­binding ­protein ­6 ­(putative)PGPL, O43824, gene:82254APEX_matrix, targetP signal+, Rickettsial homolog, mito protein domainMatrixnonenone
CYP11A1cytochrome ­P450 ­family ­11 ­subfamily ­A ­member ­1CYP11A, CYPXIA1, P450SCC, P05108, gene:15834literature, mito protein domain, MS/MS+MIMMetabolism > Lipid metabolism > Cholesterol, bile acid, steroid synthesis;
Metabolism > Metals and cofactors > Heme-containing proteins
adipose, placenta, testis
OCIAD1OCIA ­domain ­containing ­1ASRIJ, OCIA, TPA018, Q9NX40, gene:549404APEX_IMS, GFP, MS/MS++MOMnoneall 14
METTL8methyltransferase ­like ­8TIP, Q9H825, gene:798284APEX_matrix, targetP signal, Rickettsial homolog, mito protein domainMatrixMitochondrial central dogma > mtRNA metabolismnone
SMIM20small ­integral ­membrane ­protein ­20C4orf52, MITRAC7, PNX, Q8N5G0, gene:3892034literature, coexpression, MS/MSMIMOXPHOS > Complex IV > CIV assembly factors;
OXPHOS > OXPHOS assembly factors
cerebrum, cerebellum, brainstem, spinalcord, kidney, liver, heart, adipose, smallintestine, placenta, testis
RPUSD4RNA ­pseudouridine ­synthase ­D4Q96CM3, gene:848814APEX_matrix, GFP, Rickettsial homolog, mito protein domain, coexpressionMatrixMitochondrial central dogma > mtRNA metabolism > mtRNA granules;
Mitochondrial central dogma > mtRNA metabolism > mt-tRNA modifications;
Mitochondrial central dogma > mtRNA metabolism > mt-rRNA modifications
placenta
SMIM8small ­integral ­membrane ­protein ­8C6orf162, dJ102H19.2, Q96KF7, gene:571504manual, induction, coexpression, MS/MSMOMnonecerebrum, cerebellum, brainstem, spinalcord, kidney, adipose, placenta, testis
NME6NME/NM23 ­nucleoside ­diphosphate ­kinase ­6IPIA-ALPHA, NDK 6, NM23-H6, O75414, gene:102013literature, APEX_matrix, GFP, Rickettsial homolog, mito protein domain, coexpressionMatrixMetabolism > Nucleotide metabolism > Nucleotide synthesis and processinglargeintestine
FKBP8FKBP ­prolyl ­isomerase ­8FKBP38, FKBPr38, Q14318, gene:237703literature, mito protein domain, coexpression, MS/MSMOMMitochondrial dynamics and surveillance > Organelle contact sites;
Mitochondrial dynamics and surveillance > Mitophagy;
Mitochondrial dynamics and surveillance > Autophagy
cerebrum, brainstem, spinalcord, largeintestine, placenta, testis
ACSM4acyl-CoA ­synthetase ­medium ­chain ­family ­member ­4P0C7M7, gene:3413923manual, targetP signal, Rickettsial homolog, mito protein domainMatrixMetabolism > Lipid metabolism > Fatty acid oxidationnone
NLRX1NLR ­family ­member ­X1CLR11.3, DLNB26, NOD26, NOD5, NOD9, Q86UT6, gene:796713APEX_matrix, targetP signal, MS/MSMatrixSignaling > Immune responseall 14
FOXRED1FAD ­dependent ­oxidoreductase ­domain ­containing ­1FP634, H17, MC1DN19, Q96CU9, gene:555723literature, mito protein domain, coexpression+MIMOXPHOS > Complex I > CI assembly factors;
OXPHOS > OXPHOS assembly factors
adipose, largeintestine, placenta, testis
TFB2Mtranscription ­factor ­B2, ­mitochondrialHkp1, mtTFB2, Q9H5Q4, gene:642163literature, APEX_matrix, targetP signal+, mito protein domainMatrixMitochondrial central dogma > mtDNA maintenance > mtDNA replication;
Mitochondrial central dogma > mtRNA metabolism > Transcription
placenta
PPM1Kprotein ­phosphatase, ­Mg2+/Mn2+ ­dependent ­1KBDP, MSUDMV, PP2Ckappa, PP2Cm, PTMP, UG0882E07, Q8N3J5, gene:1529263literature, targetP signal+, mito protein domainMatrixMetabolism > Amino acid metabolism > BCAA metabolism;
Metabolism > Amino acid metabolism > BCAA dehydrogenase complex
cerebellum, liver, heart, adipose
ATP5MC2ATP ­synthase ­membrane ­subunit ­c ­locus ­2ATP5A, ATP5G2, Q06055, gene:5173literature, yeast mito homolog+, Rickettsial homolog, mito protein domainMIMOXPHOS > Complex V > CV subunits;
OXPHOS > OXPHOS subunits
none
MT-ND1NADH ­dehydrogenase, ­subunit ­1 ­(complex ­I)MTND1, P03886, gene:45353literature, Rickettsial homolog, mito protein domain, MS/MSMIMOXPHOS > Complex I > CI subunits;
OXPHOS > OXPHOS subunits
all 14
TAZtafazzinBTHS, CMD3A, EFE, EFE2, G4.5, LVNCX, Taz1, Q16635, gene:69013literature, yeast mito homolog++, mito protein domainIMSMetabolism > Lipid metabolism > Cardiolipin synthesisnone
MSRB3methionine ­sulfoxide ­reductase ­B3DFNB74, Q8IXL7, gene:2538273literature, yeast mito homolog+, mito protein domainunknownMetabolism > Detoxification > ROS and glutathione metabolismnone
ACOT11acyl-CoA ­thioesterase ­11BFIT, STARD14, THEA, THEM1, Q8WXI4, gene:260273literature, targetP signal, mito protein domain, coexpressionMatrixMetabolism > Lipid metabolism > Fatty acid oxidationnone
NIPSNAP3Bnipsnap ­homolog ­3BFP944, NIPSNAP3, SNAP1, Q9BS92, gene:553353GFP, targetP signal, MS/MS+Matrixnonecerebrum, cerebellum, brainstem, spinalcord, kidney, liver, adipose, smallintestine, largeintestine, stomach, placenta, testis
NTHL1nth ­like ­DNA ­glycosylase ­1FAP3, NTH1, OCTS3, hNTH1, P78549, gene:49133literature, yeast mito homolog, Rickettsial homolog, mito protein domainunknownMetabolism > Metals and cofactors > Fe-S-containing proteinsnone
MARCHF5membrane ­associated ­ring-CH-type ­finger ­5MARCH-V, MARCH5, MITOL, RNF153, Q9NX47, gene:547083literature, mito protein domain, coexpression, MS/MSMOMMitochondrial dynamics and surveillance > Mitophagy;
Mitochondrial dynamics and surveillance > Autophagy
cerebrum, cerebellum, brainstem, spinalcord, liver, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
SFXN4sideroflexin ­4BCRM1, COXPD18, SLC56A4, Q6P4A7, gene:1195593literature, mito protein domain+MIMSmall molecule transport > Sideroflexinscerebellum, spinalcord
TRMT5tRNA ­methyltransferase ­5COXPD26, KIAA1393, TRM5, Q32P41, gene:575703literature, yeast mito homolog, mito protein domain, coexpressionMatrixMitochondrial central dogma > mtRNA metabolism > mt-tRNA modificationsnone
SLC25A6solute ­carrier ­family ­25 ­member ­6AAC3, ANT, ANT 2, ANT 3, ANT3, ANT3Y, P12236, gene:2933literature, yeast mito homolog+, mito protein domain+MIMMetabolism > Nucleotide metabolism > Nucleotide import;
Signaling > Calcium homeostasis > Mitochondrial permeability transition pore;
Small molecule transport > SLC25A family
none
SLC25A52solute ­carrier ­family ­25 ­member ­52MCART2, Q3SY17, gene:1474073manual, yeast mito homolog+, mito protein domain+MIMMetabolism > Metals and cofactors > NAD biosynthesis and metabolism;
Small molecule transport > SLC25A family
none
OGG18-oxoguanine ­DNA ­glycosylaseHMMH, HOGG1, MUTM, OGH1, O15527, gene:49683literature, targetP signal, yeast mito homolog, mito protein domainMatrixMitochondrial central dogma > mtDNA maintenance > mtDNA repairnone
ACOD1aconitate ­decarboxylase ­1CAD, IRG1, A6NK06, gene:7302493literature, yeast mito homolog, mito protein domain+MatrixMetabolism > Carbohydrate metabolism > Itaconate metabolism;
Signaling > Immune response
none
QTRT1queuine ­tRNA-ribosyltransferase ­catalytic ­subunit ­1FP3235, TGT, TGUT, Q9BXR0, gene:818903APEX_matrix, targetP signal, Rickettsial homolog, mito protein domain, coexpressionMatrixMitochondrial central dogma > mtRNA metabolism > mt-tRNA modificationsnone
AIFM2apoptosis ­inducing ­factor ­mitochondria ­associated ­2AMID, FSP1, PRG3, Q9BRQ8, gene:848833literature, Rickettsial homolog, mito protein domain, coexpressionMembraneMetabolism > Detoxification > ROS and glutathione metabolism;
Mitochondrial dynamics and surveillance > Apoptosis
none
PRDX2peroxiredoxin ­2HEL-S-2a, NKEF-B, NKEFB, PRP, PRX2, PRXII, PTX1, TDPX1, TPX1, TSA, P32119, gene:70013GFP, Rickettsial homolog, mito protein domain, coexpressionunknownMetabolism > Detoxification > ROS and glutathione metabolismcerebrum, cerebellum, brainstem, spinalcord, adipose, smallintestine, stomach, placenta, testis
FBXL4F-box ­and ­leucine ­rich ­repeat ­protein ­4FBL4, FBL5, MTDPS13, Q9UKA2, gene:262352literature, mito protein domain, coexpressionIMSnoneadipose
GPAMglycerol-3-phosphate ­acyltransferase, ­mitochondrialGPAT, GPAT1, Q9HCL2, gene:576782literature, mito protein domain, coexpressionMembraneMetabolism > Lipid metabolism > Cardiolipin synthesis;
Metabolism > Lipid metabolism > Phospholipid metabolism
cerebellum, spinalcord, kidney, liver, skeletalmuscle, adipose, largeintestine, stomach, placenta, testis
DMAC2Ldistal ­membrane ­arm ­assembly ­complex ­2 ­likeATP5S, ATPW, FB, HSU79253, Q99766, gene:271092literature, APEX_matrix, MS/MS++MIMOXPHOS > Complex V > CV subunits;
OXPHOS > OXPHOS subunits
cerebrum, cerebellum, brainstem, spinalcord, kidney, liver, heart, skeletalmuscle, adipose, largeintestine, stomach, placenta, testis
RMDN3regulator ­of ­microtubule ­dynamics ­3FAM82A2, FAM82C, RMD-3, RMD3, ptpip51, Q96TC7, gene:551772literature, APEX_IMS, GFP, MS/MS++MOMMitochondrial dynamics and surveillance > Organelle contact sitescerebrum, cerebellum, brainstem, spinalcord, kidney, liver, adipose, largeintestine, stomach, placenta, testis
PRXL2Aperoxiredoxin ­like ­2AAdrx, C10orf58, FAM213A, PAMM, Q9BRX8, gene:842932GFP, MS/MS++MembraneMetabolism > Detoxification > ROS and glutathione metabolismcerebrum, cerebellum, brainstem, spinalcord, kidney, liver, skeletalmuscle, adipose, smallintestine, placenta, testis
ATP5MDATP ­synthase ­membrane ­subunit ­DAPITDAPIT, HCVFTP2, MC5DN6, USMG5, bA792D24.4, Q96IX5, gene:848332GFP, MS/MS++MIMOXPHOS > Complex V > CV subunits;
OXPHOS > OXPHOS subunits;
Mitochondrial dynamics and surveillance > Cristae formation
all 14
PET117PET117 ­cytochrome ­c ­oxidase ­chaperoneCSRP2BP, Q6UWS5, gene:1003037552literature, yeast mito homolog, MS/MSMIMOXPHOS > Complex IV > CIV assembly factors;
OXPHOS > OXPHOS assembly factors
cerebrum, cerebellum, liver, adipose, largeintestine, stomach, placenta
MAOAmonoamine ­oxidase ­ABRNRS, MAO-A, P21397, gene:41282literature, mito protein domain, MS/MS+MOMMetabolism > Amino acid metabolism > Catechol metabolism;
Metabolism > Detoxification > Xenobiotic metabolism
all 14
C15orf61chromosome ­15 ­open ­reading ­frame ­61A6NNL5, gene:1458532APEX_matrix, targetP signal, MS/MSMatrixnonebrainstem, spinalcord, skeletalmuscle, adipose, largeintestine, stomach, testis
POLQDNA ­polymerase ­thetaPRO0327, O75417, gene:107212literature, targetP signal, Rickettsial homolog, mito protein domainMatrixMitochondrial central dogma > mtDNA maintenancebrainstem, testis
BCO2beta-carotene ­oxygenase ­2B-DIOX-II, BCDO2, Q9BYV7, gene:838752literature, targetP signal, mito protein domain, MS/MSMIMMetabolism > Vitamin metabolism > Vitamin A metabolismsmallintestine
IFI27interferon ­alpha ­inducible ­protein ­27FAM14D, ISG12, ISG12A, P27, P40305, gene:34292literature, induction, coexpressionMembraneSignaling > Immune response;
Mitochondrial dynamics and surveillance > Apoptosis
none
CRYZcrystallin ­zetaQ08257, gene:14292APEX_matrix, mito protein domain, MS/MSMatrixnonespinalcord, kidney, liver, adipose, placenta, testis
PYCR1pyrroline-5-carboxylate ­reductase ­1ARCL2B, ARCL3B, P5C, P5CR, PIG45, PP222, PRO3, PYCR, P32322, gene:58312literature, mito protein domain, MS/MSMatrixMetabolism > Amino acid metabolism > Proline metabolismcerebrum, cerebellum, brainstem, spinalcord, adipose, largeintestine, stomach, placenta, testis
TMLHEtrimethyllysine ­hydroxylase, ­epsilonAUTSX6, BBOX2, TMLD, TMLH, TMLHED, XAP130, Q9NVH6, gene:552172literature, APEX_matrix, mito protein domain, MS/MSMatrixMetabolism > Metals and cofactors > Carnitine synthesis and transportkidney, adipose, smallintestine, largeintestine, stomach, placenta, testis
TOMM20translocase ­of ­outer ­mitochondrial ­membrane ­20MAS20, MOM19, TOM20, Q15388, gene:98042literature, yeast mito homolog+MOMProtein import, sorting and homeostasis > Protein import and sorting > TOM;
Mitochondrial dynamics and surveillance
cerebrum, cerebellum, brainstem, spinalcord, kidney, liver, heart, skeletalmuscle, adipose, largeintestine, stomach, placenta, testis
YRDCyrdC ­N6-threonylcarbamoyltransferase ­domain ­containingDRIP3, IRIP, SUA5, Q86U90, gene:796932manual, targetP signal+, Rickettsial homologMatrixMitochondrial central dogma > mtRNA metabolism > mt-tRNA modificationsnone
COA7cytochrome ­c ­oxidase ­assembly ­factor ­7 ­(putative)C1orf163, RESA1, SCAN3, SELRC1, Q96BR5, gene:652602literature, APEX_IMS, mito protein domain, coexpression, MS/MSIMSOXPHOS > Complex IV > CIV assembly factors;
OXPHOS > OXPHOS assembly factors
cerebrum, cerebellum, brainstem, spinalcord, kidney, liver, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
DHRS2dehydrogenase/reductase ­2HEP27, SDR25C1, Q13268, gene:102022literature, targetP signal, Rickettsial homolog, mito protein domainMatrixMetabolism > Detoxification > Xenobiotic metabolismnone
MMAAmetabolism ­of ­cobalamin ­associated ­AcblA, Q8IVH4, gene:1667852manual, coexpression+MatrixMetabolism > Vitamin metabolism > Vitamin B12 metabolismcerebrum, cerebellum, brainstem, kidney, liver, heart, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
MIEF1mitochondrial ­elongation ­factor ­1AltMIEF1, HSU79252, MID51, MIEF1-MP, SMCR7L, dJ1104E15.3, Q9NQG6, gene:544712literature, mito protein domain, induction, coexpressionMOM, MatrixMitochondrial central dogma > Translation > Mitochondrial ribosome assembly;
Mitochondrial dynamics and surveillance > Fission
none
C15orf48chromosome ­15 ­open ­reading ­frame ­48COXFA4L3, FOAP-11, MIR147BHG, NMES1, Q9C002, gene:844191GFP, mito protein domain, MS/MS+Membranenonesmallintestine, largeintestine, stomach, placenta, testis
LIG3DNA ­ligase ­3LIG2, LIG3alpha, P49916, gene:39801literature, targetP signal, mito protein domain, coexpressionMatrixMitochondrial central dogma > mtDNA maintenance > mtDNA replication;
Mitochondrial central dogma > mtDNA maintenance > mtDNA repair
none
BNIP3LBCL2 ­interacting ­protein ­3 ­likeBNIP3a, NIX, O60238, gene:6651literature, mito protein domain+, coexpressionMOMMitochondrial dynamics and surveillance > Apoptosisnone
FASTKD5FAST ­kinase ­domains ­5dJ1187M17.5, Q7L8L6, gene:604931APEX_matrix, targetP signal, coexpressionMatrixMitochondrial central dogma > mtRNA metabolism > mtRNA granules;
Mitochondrial central dogma > mtRNA metabolism > Polycistronic mtRNA processing;
Mitochondrial central dogma > mtRNA metabolism > mtRNA stability and decay
skeletalmuscle, largeintestine, placenta
PARK7Parkinsonism ­associated ­deglycaseDJ-1, DJ1, GATD2, HEL-S-67p, Q99497, gene:113151literature, mito protein domain, coexpressionMOMProtein import, sorting and homeostasis > Protein homeostasis;
Mitochondrial dynamics and surveillance > Mitophagy;
Mitochondrial dynamics and surveillance > Autophagy
all 14
COA1cytochrome ­c ­oxidase ­assembly ­factor ­1 ­homologC7orf44, MITRAC15, Q9GZY4, gene:557441APEX_IMS, targetP signal, mito protein domain+MIMOXPHOS > Complex I > CI assembly factors;
OXPHOS > Complex IV > CIV assembly factors;
OXPHOS > OXPHOS assembly factors
none
COX7B2cytochrome ­c ­oxidase ­subunit ­7B2Q8TF08, gene:1707121literature, targetP signal, mito protein domain+MIMOXPHOS > Complex IV > CIV subunits;
OXPHOS > OXPHOS subunits
none
MT-ND4LNADH ­dehydrogenase, ­subunit ­4L ­(complex ­I)MTND4L, P03901, gene:45391literature, Rickettsial homolog, mito protein domainMIMOXPHOS > Complex I > CI subunits;
OXPHOS > OXPHOS subunits
heart, skeletalmuscle
SIRT4sirtuin ­4SIR2L4, Q9Y6E7, gene:234091literature, targetP signal, mito protein domainMatrixMetabolism > Metals and cofactors > NAD biosynthesis and metabolismlargeintestine
UQCC2ubiquinol-cytochrome ­c ­reductase ­complex ­assembly ­factor ­2C6orf125, C6orf126, Cbp6, M19, MC3DN7, MNF1, bA6B20.2, Q9BRT2, gene:843001APEX_matrix, GFP, coexpression, MS/MSMIMOXPHOS > Complex III > CIII assembly factors;
OXPHOS > OXPHOS assembly factors
all 14
USP30ubiquitin ­specific ­peptidase ­30Q70CQ3, gene:847491literature, targetP signal, mito protein domainMOMProtein import, sorting and homeostasis > Protein homeostasis > Proteases;
Mitochondrial dynamics and surveillance > Mitophagy;
Mitochondrial dynamics and surveillance > Autophagy
adipose, placenta, testis
PDE12phosphodiesterase ­122'-PDE, 2-PDE, Q6L8Q7, gene:2016261APEX_matrix, targetP signal, mito protein domainMatrixMitochondrial central dogma > mtRNA metabolism > mt-mRNA modifications;
Mitochondrial central dogma > mtRNA metabolism > mtRNA stability and decay
brainstem, smallintestine, largeintestine, testis
NME3NME/NM23 ­nucleoside ­diphosphate ­kinase ­3DR-nm23, NDPK-C, NDPKC, NM23-H3, NM23H3, c371H6.2, Q13232, gene:48321GFP, Rickettsial homolog, mito protein domainMOMMetabolism > Nucleotide metabolism > Nucleotide synthesis and processingcerebrum, cerebellum, brainstem, kidney, adipose, placenta, testis
DHX30DExH-box ­helicase ­30DDX30, NEDMIAL, RETCOR, Q7L2E3, gene:229071literature, mito protein domainMatrixMitochondrial central dogma > mtRNA metabolism > mtRNA granules;
Mitochondrial central dogma > Translation > Mitochondrial ribosome assembly
cerebrum, cerebellum, brainstem, spinalcord, kidney, liver, skeletalmuscle, adipose, largeintestine, stomach, placenta, testis
FASTKFas ­activated ­serine/threonine ­kinaseFAST, Q14296, gene:109221GFP, induction, coexpressionMIMMitochondrial central dogma > mtRNA metabolism > mtRNA granules;
Mitochondrial central dogma > mtRNA metabolism > Polycistronic mtRNA processing;
Mitochondrial central dogma > mtRNA metabolism > mtRNA stability and decay
none
METTL15methyltransferase ­like ­15METT5D1, A6NJ78, gene:1960741APEX_matrix, Rickettsial homolog, coexpressionMatrixMitochondrial central dogma > mtRNA metabolism > mt-rRNA modificationslargeintestine, placenta
CYP24A1cytochrome ­P450 ­family ­24 ­subfamily ­A ­member ­1CP24, CYP24, HCAI, HCINF1, P450-CC24, Q07973, gene:15911literature, targetP signal+, mito protein domainMIMMetabolism > Metals and cofactors > Heme-containing proteins;
Metabolism > Vitamin metabolism > Vitamin D metabolism
none
NOCTnocturninCCR4L, CCRN4L, Ccr4c, NOC, Q9UK39, gene:258191literature, targetP signal+, mito protein domainMatrixMetabolism > Metals and cofactors > NAD biosynthesis and metabolismnone
PANK2pantothenate ­kinase ­2C20orf48, HARP, HSS, NBIA1, PKAN, Q9BZ23, gene:800251literature, targetP signal+, mito protein domainIMSMetabolism > Metals and cofactors > Coenzyme A metabolismnone
CA5Acarbonic ­anhydrase ­5ACA5, CA5AD, CAV, CAVA, GS1-21A4.1, P35218, gene:7631literature, mito protein domain, MS/MSMatrixMetabolism > Amino acid metabolism > Urea cycleliver
NT5DC25'-nucleotidase ­domain ­containing ­2Q9H857, gene:649431APEX_matrix, targetP signal, MS/MSMatrixMetabolism > Nucleotide metabolism > Nucleotide synthesis and processingspinalcord, largeintestine, placenta
ATAD3BATPase ­family ­AAA ­domain ­containing ­3BAAA-TOB3, TOB3, Q5T9A4, gene:838581APEX_IMS, APEX_matrix, GFP, Rickettsial homolog, mito protein domain+MIMMitochondrial central dogma > mtDNA maintenance > mtDNA nucleoid;
Mitochondrial dynamics and surveillance > Organelle contact sites;
Mitochondrial dynamics and surveillance > Intramitochondrial membrane interactions
none
TRUB2TruB ­pseudouridine ­synthase ­family ­member ­2CLONE24922, O95900, gene:269951APEX_matrix, Rickettsial homolog, mito protein domainMatrixMitochondrial central dogma > mtRNA metabolism > mtRNA granules;
Mitochondrial central dogma > mtRNA metabolism > mt-rRNA modifications
none
PRKACAprotein ­kinase ­cAMP-activated ­catalytic ­subunit ­alphaPKACA, PPNAD4, P17612, gene:55660literature, mito protein domain, coexpressionMIMSignaling > cAMP-PKA signalingcerebrum, brainstem, spinalcord, adipose, testis
COMTD1catechol-O-methyltransferase ­domain ­containing ­1Q86VU5, gene:1188810GFP, MS/MS++Membranenoneall 14
CPT1Ccarnitine ­palmitoyltransferase ­1CCATL1, CPT1-B, CPT1P, CPTI-B, CPTIC, SPG73, Q8TCG5, gene:1261290literature, yeast mito homolog+, mito protein domainMOMMetabolism > Lipid metabolism > Fatty acid oxidation;
Metabolism > Metals and cofactors > Carnitine synthesis and transport;
Metabolism > Metals and cofactors > Carnitine shuttle
none
C3orf33chromosome ­3 ­open ­reading ­frame ­33AC3-33, Q6P1S2, gene:2853150APEX_matrix, targetP signal, MS/MSMatrixnoneadipose, testis
MIGA2mitoguardin ­2C9orf54, FAM73B, Q7L4E1, gene:848950literature, coexpressionMOMMitochondrial dynamics and surveillance > Fusion;
Mitochondrial dynamics and surveillance > Organelle contact sites
cerebrum, cerebellum, brainstem, spinalcord, liver, testis
SND1staphylococcal ­nuclease ­and ­tudor ­domain ­containing ­1TDRD11, Tudor-SN, p100, Q7KZF4, gene:270440GFP, yeast mito homolog, mito protein domainunknownnonecerebrum, cerebellum, brainstem, liver, smallintestine, placenta, testis
GLUD2glutamate ­dehydrogenase ­2GDH2, GLUDP1, P49448, gene:27470literature, targetP signal+, mito protein domainMatrixMetabolism > Amino acid metabolism > Glutamate metabolism;
Metabolism > Amino acid metabolism > GABA metabolism
none
HPDL4-hydroxyphenylpyruvate ­dioxygenase ­like4-HPPD-L, GLOXD1, Q96IR7, gene:848420manual, targetP signal, inductionunknownnonenone
ANGEL2angel ­homolog ­2Ccr4d, KIAA0759L, Q5VTE6, gene:908060APEX_matrix, mito protein domain, MS/MSMatrixMitochondrial central dogma > mtRNA metabolism > mt-mRNA modificationsadipose, smallintestine, placenta, testis
ANTKMTadenine ­nucleotide ­translocase ­lysine ­methyltransferaseANT-KMT, C16orf24, FAM173A, Q9BQD7, gene:659900literature, coexpression, MS/MSMIMMetabolism > Nucleotide metabolismcerebrum, cerebellum, brainstem, spinalcord, adipose, largeintestine, placenta
UQCC3ubiquinol-cytochrome ­c ­reductase ­complex ­assembly ­factor ­3C11orf83, CCDS41658.1, MC3DN9, UNQ655, Q6UW78, gene:7909550literature, coexpression, MS/MSMIMOXPHOS > Complex III > CIII assembly factors;
OXPHOS > OXPHOS assembly factors
liver, skeletalmuscle, adipose, largeintestine, stomach, placenta, testis
PRDX4peroxiredoxin ­4AOE37-2, AOE372, HEL-S-97n, PRX-4, Q13162, gene:105490APEX_IMS, Rickettsial homolog, mito protein domainIMSMetabolism > Detoxification > ROS and glutathione metabolismcerebrum, cerebellum, brainstem, spinalcord, kidney, liver, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
CDK5RAP1CDK5 ­regulatory ­subunit ­associated ­protein ­1C20orf34, C42, CGI-05, HSPC167, Q96SZ6, gene:516540literature, targetP signal, Rickettsial homolog, mito protein domainMatrixMitochondrial central dogma > mtRNA metabolism > mt-tRNA modificationsnone
PAICSphosphoribosylaminoimidazole ­carboxylase ­and ­phosphoribosylaminoimidazolesuccinocarboxamide ­synthaseADE2, ADE2H1, AIRC, PAIS, P22234, gene:106060APEX_matrix, Rickettsial homolog, coexpressionMatrixMetabolism > Nucleotide metabolism > Nucleotide synthesis and processingnone
MGARPmitochondria ­localized ­glutamic ­acid ­rich ­proteinC4orf49, CESP-1, HUMMR, OSAP, Q8TDB4, gene:847090literature, APEX_IMS, targetP signal, coexpressionMembraneMitochondrial dynamics and surveillance > Traffickingnone
HDHD3haloacid ­dehalogenase ­like ­hydrolase ­domain ­containing ­32810435D12Rik, C9orf158, Q9BSH5, gene:819320APEX_matrix, MS/MS++Matrixnonecerebrum, cerebellum, brainstem, spinalcord, kidney, liver, adipose, smallintestine, largeintestine, stomach, placenta, testis
CYP11B2cytochrome ­P450 ­family ­11 ­subfamily ­B ­member ­2ALDOS, CPN2, CYP11B, CYP11BL, CYPXIB2, P-450C18, P450C18, P450aldo, P19099, gene:15850literature, targetP signal, mito protein domain, coexpressionMIMMetabolism > Lipid metabolism > Cholesterol, bile acid, steroid synthesis;
Metabolism > Metals and cofactors > Heme-containing proteins
none
AGPAT41-acylglycerol-3-phosphate ­O-acyltransferase ­41-AGPAT4, LPAAT-delta, dJ473J16.2, Q9NRZ5, gene:568950literature, mito protein domain, inductionMOMMetabolism > Lipid metabolism > Cardiolipin synthesis;
Metabolism > Lipid metabolism > Phospholipid metabolism
testis
RCC1LRCC1 ­likeWBSCR16, Q96I51, gene:81554-0literature, APEX_matrix, targetP signal, coexpressionMatrixMitochondrial central dogma > mtRNA metabolism > mt-rRNA modificationsplacenta
FAM185Afamily ­with ­sequence ­similarity ­185 ­member ­AQ8N0U4, gene:222234-0APEX_matrix, targetP signal, coexpressionMatrixnonenone
PMAIP1phorbol-12-myristate-13-acetate-induced ­protein ­1APR, NOXA, Q13794, gene:5366-0literature, mito protein domain+MOMMitochondrial dynamics and surveillance > Apoptosisnone
TDRKHtudor ­and ­KH ­domain ­containingTDRD2, Q9Y2W6, gene:11022-0GFP, mito protein domain, MS/MSMembranenonecerebrum, cerebellum, brainstem, spinalcord, adipose, testis
SLC8B1solute ­carrier ­family ­8 ­member ­B1NCKX6, NCLX, SLC24A6, Q6J4K2, gene:80024-0literature, mito protein domain, inductionMIMSignaling > Calcium homeostasis > Calcium cycle;
Small molecule transport
placenta
CYP27B1cytochrome ­P450 ­family ­27 ­subfamily ­B ­member ­1CP2B, CYP1, CYP1alpha, CYP27B, P450c1, PDDR, VDD1, VDDR, VDDRI, VDR, O15528, gene:1594-1literature, targetP signal, mito protein domainMIMMetabolism > Metals and cofactors > Heme-containing proteins;
Metabolism > Vitamin metabolism > Vitamin D metabolism
none
POLG2DNA ­polymerase ­gamma ­2, ­accessory ­subunitHP55, MTDPS16, MTPOLB, PEOA4, POLB, POLG-BETA, POLGB, Q9UHN1, gene:11232-1literature, APEX_matrix, targetP signal, mito protein domainMatrixMitochondrial central dogma > mtDNA maintenance > mtDNA replication;
Mitochondrial central dogma > mtDNA maintenance > mtDNA nucleoid
none
THEM5thioesterase ­superfamily ­member ­5ACOT15, Q8N1Q8, gene:284486-1literature, targetP signal, mito protein domainMatrixMetabolism > Lipid metabolismnone
BIKBCL2 ­interacting ­killerBIP1, BP4, NBK, Q13323, gene:638-1manual, mito protein domain+MOMMitochondrial dynamics and surveillance > Apoptosisnone
MTERF1mitochondrial ­transcription ­termination ­factor ­1MTERF, Q99551, gene:7978-1literature, APEX_matrix, mito protein domain+MatrixMitochondrial central dogma > mtDNA maintenance > mtDNA nucleoid;
Mitochondrial central dogma > mtRNA metabolism > Transcription
none
MTFR2mitochondrial ­fission ­regulator ­2DUFD1, FAM54A, Q6P444, gene:113115-1manual, mito protein domain+MembraneMitochondrial dynamics and surveillance > Fissionnone
GPX4glutathione ­peroxidase ­4GPx-4, GSHPx-4, MCSP, PHGPx, SMDS, snGPx, snPHGPx, P36969, gene:2879-1literature, mito protein domainMIMMetabolism > Detoxification > ROS and glutathione metabolism;
Metabolism > Detoxification > Selenoproteins
all 14
TOMM20Ltranslocase ­of ­outer ­mitochondrial ­membrane ­20 ­likeUNQ9438, Q6UXN7, gene:387990-1manual, yeast mito homolog+MOMProtein import, sorting and homeostasis > Protein import and sortingnone
COMTcatechol-O-methyltransferaseHEL-S-98n, P21964, gene:1312-1GFP, induction, coexpressionMembraneMetabolism > Amino acid metabolism > Catechol metabolismliver
MYG1MYG1 ­exonucleaseC12orf10, Gamm1, MST024, MSTP024, MYG, Q86UA3, gene:60314-1literature, mito protein domain, coexpressionMatrixMitochondrial central dogma > mtRNA metabolism > mt-rRNA modifications;
Mitochondrial central dogma > mtRNA metabolism > mt-mRNA modifications
none
SPATA20spermatogenesis ­associated ­20HEL-S-98, SSP411, Tisp78, Q8TB22, gene:64847-1APEX_matrix, targetP signalMatrixnonetestis
OXCT23-oxoacid ­CoA-transferase ­2FKSG25, SCOTT, Q9BYC2, gene:64064-1manual, targetP signal+, mito protein domainMatrixMetabolism > Carbohydrate metabolism > Ketone metabolismnone
CCDC127coiled-coil ­domain ­containing ­127Q96BQ5, gene:133957-1APEX_IMS, MS/MSMembranenonecerebrum, cerebellum, brainstem, spinalcord, liver, heart, adipose, smallintestine, largeintestine, stomach, placenta, testis
CRY1cryptochrome ­circadian ­regulator ­1DSPD, PHLL1, Q16526, gene:1407-1literature, mito protein domain, coexpressionunknownnonenone
ACSM2Bacyl-CoA ­synthetase ­medium ­chain ­family ­member ­2BACSM2, HXMA, HYST1046, Q68CK6, gene:348158-1manual, targetP signal, mito protein domainMatrixMetabolism > Lipid metabolism > Fatty acid oxidationnone
MYO19myosin ­XIXMYOHD1, Q96H55, gene:80179-1literature, mito protein domainMOMMitochondrial dynamics and surveillance > Traffickingadipose
MGME1mitochondrial ­genome ­maintenance ­exonuclease ­1C20orf72, DDK1, MTDPS11, bA504H3.4, Q9BQP7, gene:92667-2literature, APEX_matrix, GFP, targetP signalMatrixMitochondrial central dogma > mtDNA maintenance > mtDNA replication;
Mitochondrial central dogma > mtDNA maintenance > mtDNA stability and decay
largeintestine, placenta
SEPTIN4septin ­4ARTS, BRADEION, C17orf47, CE5B3, H5, MART, PNUTL2, SEP4, SEPT4, hCDCREL-2, hucep-7, O43236, gene:5414-2literature, mito protein domainMOMMitochondrial dynamics and surveillance > Apoptosisbrainstem, spinalcord
EFHD1EF-hand ­domain ­family ­member ­D1MST133, MSTP133, PP3051, SWS2, Q9BUP0, gene:80303-2literature, mito protein domain, inductionMIMSignaling > Calcium homeostasis > EF hand proteinsnone
RAB5IFRAB5 ­interacting ­factorC20orf24, PNAS-11, RCAF1, RIP5, Q9BUV8, gene:55969-2GFP, induction, coexpressionMIMOXPHOS > OXPHOS assembly factors;
OXPHOS > Respirasome assembly
none
APEX1apurinic/apyrimidinic ­endodeoxyribonuclease ­1APE, APE1, APEN, APEX, APX, HAP1, REF1, P27695, gene:328-2literature, Rickettsial homolog, mito protein domainMatrixMitochondrial central dogma > mtDNA maintenance > mtDNA repairnone
SPTLC2serine ­palmitoyltransferase ­long ­chain ­base ­subunit ­2HSN1C, LCB2, LCB2A, NSAN1C, SPT2, hLCB2a, O15270, gene:9517-2GFP, Rickettsial homolog, mito protein domainMembraneMetabolism > Lipid metabolism > Phospholipid metabolismnone
DMAC1distal ­membrane ­arm ­assembly ­complex ­1C9orf123, TMEM261, Q96GE9, gene:90871-2literature, MS/MSMIMOXPHOS > Complex I > CI assembly factors;
OXPHOS > OXPHOS assembly factors
cerebrum, spinalcord, kidney, heart, adipose, smallintestine, largeintestine, stomach, placenta, testis
ATPSCKMTATP ­synthase ­c ­subunit ­lysine ­N-methyltransferaseFAM173B, JS-2, hFAM173B, Q6P4H8, gene:134145-2literature, coexpressionMIMOXPHOS > Complex V > CV assembly factors;
OXPHOS > OXPHOS assembly factors
placenta
NEU4neuraminidase ­4Q8WWR8, gene:129807-2GFP, targetP signalMOMMetabolism > Carbohydrate metabolismnone
CASP9caspase ­9APAF-3, APAF3, ICE-LAP6, MCH6, PPP1R56, P55211, gene:842-2literature, targetP signal, mito protein domainIMSProtein import, sorting and homeostasis > Protein homeostasis > Proteases;
Mitochondrial dynamics and surveillance > Apoptosis
none
AGKacylglycerol ­kinaseCATC5, CTRCT38, MTDPS10, MULK, Q53H12, gene:55750-2literature, APEX_IMS, mito protein domainMIMProtein import, sorting and homeostasis > Protein import and sorting > TIM22 carrier pathway;
Metabolism > Lipid metabolism > Phospholipid metabolism
cerebrum, cerebellum, brainstem, spinalcord, liver, heart, adipose, smallintestine, largeintestine, stomach, placenta, testis
MCL1MCL1 ­apoptosis ­regulator, ­BCL2 ­family ­memberBCL2L3, EAT, MCL1-ES, MCL1L, MCL1S, Mcl-1, TM, bcl2-L-3, mcl1/EAT, Q07820, gene:4170-3manual, mito protein domainMOMMitochondrial dynamics and surveillance > Apoptosisadipose, largeintestine, placenta, testis
DUS2dihydrouridine ­synthase ­2DUS2L, SMM1, URLC8, Q9NX74, gene:54920-3GFP, Rickettsial homolog, mito protein domainMatrixMitochondrial central dogma > mtRNA metabolism > mt-tRNA modificationsnone
PLD6phospholipase ­D ­family ­member ­6ZUC, Q8N2A8, gene:201164-3literature, Rickettsial homolog, mito protein domainMOMMetabolism > Lipid metabolism > Cardiolipin synthesis;
Mitochondrial dynamics and surveillance > Fusion
none
TOMM34translocase ­of ­outer ­mitochondrial ­membrane ­34HTOM34P, TOM34, URCC3, Q15785, gene:10953-3literature, mito protein domainMOMProtein import, sorting and homeostasis > Protein import and sorting > TOMtestis
PRORPprotein ­only ­RNase ­P ­catalytic ­subunitKIAA0391, MRPP3, O15091, gene:9692-3APEX_matrixMatrixMitochondrial central dogma > mtRNA metabolism > mtRNA granules;
Mitochondrial central dogma > mtRNA metabolism > Polycistronic mtRNA processing
spinalcord, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
PLSCR3phospholipid ­scramblase ­3Q9NRY6, gene:57048-3literature, mito protein domainMIMMetabolism > Lipid metabolism > Cardiolipin synthesisnone
CKMT1Bcreatine ­kinase, ­mitochondrial ­1BCKMT, CKMT1, UMTCK, 0, gene:1159-3literature, APEX_IMS, targetP signal, mito protein domainMIMMetabolism > Nucleotide metabolism > Creatine metabolismnone
NDUFB1NADH:ubiquinone ­oxidoreductase ­subunit ­B1CI-MNLL, CI-SGDH, MNLL, O75438, gene:4707-3literature, mito protein domain+MIMOXPHOS > Complex I > CI subunits;
OXPHOS > OXPHOS subunits
none
ATP5MF-PTCD1ATP5MF-PTCD1 ­readthroughATP5J2-PTCD1, B4DJ38, gene:100526740-3APEX_matrix, mito protein domain+unknownnonenone
CASP8caspase ­8ALPS2B, CAP4, Casp-8, FLICE, MACH, MCH5, Q14790, gene:841-3literature, mito protein domainMOMProtein import, sorting and homeostasis > Protein homeostasis;
Mitochondrial dynamics and surveillance > Apoptosis
none
BCL2L1BCL2 ­like ­1BCL-XL/S, BCL2L, BCLX, Bcl-X, PPP1R52, Q07817, gene:598-3literature, mito protein domainMOMMitochondrial dynamics and surveillance > Apoptosiscerebrum, cerebellum, placenta
EXD2exonuclease ­3'-5' ­domain ­containing ­2C14orf114, EXDL2, Q9NVH0, gene:55218-3literature, mito protein domainMatrixMitochondrial central dogma > Translationcerebrum, cerebellum, testis
ARMC10armadillo ­repeat ­containing ­10PNAS-112, PNAS112, PSEC0198, SVH, Q8N2F6, gene:83787-4GFP, MS/MSMOMMitochondrial dynamics and surveillance > Fission;
Mitochondrial dynamics and surveillance > Trafficking
cerebrum, cerebellum, brainstem, spinalcord, liver, adipose, largeintestine, stomach, placenta, testis
MIEF2mitochondrial ­elongation ­factor ­2MID49, SMCR7, Q96C03, gene:125170-4literature, mito protein domainMOMMitochondrial dynamics and surveillance > Fissionnone
THG1LtRNA-histidine ­guanylyltransferase ­1 ­likeICF45, IHG-1, IHG1, SCAR28, THG1, hTHG1, Q9NWX6, gene:54974-4literature, targetP signal, coexpressionMatrixMitochondrial central dogma > mtRNA metabolism > mt-tRNA modificationsnone
GOLPH3golgi ­phosphoprotein ­3GOPP1, GPP34, MIDAS, Vps74, Q9H4A6, gene:64083-4literature, inductionIMSnonenone
PREPLprolyl ­endopeptidase ­likeCMS22, Q4J6C6, gene:9581-4APEX_matrix, Rickettsial homologMatrixnonespinalcord, largeintestine
FASTKD3FAST ­kinase ­domains ­3Q14CZ7, gene:79072-4APEX_matrix, targetP signalMatrixMitochondrial central dogma > mtRNA metabolism > Polycistronic mtRNA processing;
Mitochondrial central dogma > mtRNA metabolism > mtRNA stability and decay
none
CEP89centrosomal ­protein ­89CCDC123, CEP123, Q96ST8, gene:84902-4literature, targetP signalIMSOXPHOS > Complex IV > CIV assembly factors;
OXPHOS > OXPHOS assembly factors
none
GPAT2glycerol-3-phosphate ­acyltransferase ­2, ­mitochondrialCT123, Q6NUI2, gene:150763-5manual MOMMetabolism > Lipid metabolism > Cardiolipin synthesis;
Metabolism > Lipid metabolism > Phospholipid metabolism
testis
NBR1NBR1 ­autophagy ­cargo ­receptor1A1-3B, IAI3B, M17S2, MIG19, Q14596, gene:4077-5APEX_IMS, coexpressionIMSMitochondrial dynamics and surveillance > Autophagycerebellum, liver
BOKBCL2 ­family ­apoptosis ­regulator ­BOKBCL2L9, BOKL, Q9UMX3, gene:666-5manual, mito protein domainMOMMitochondrial dynamics and surveillance > Apoptosisnone
FKBP10FKBP ­prolyl ­isomerase ­10BRKS1, FKBP65, OI11, OI6, PPIASE, hFKBP65, Q96AY3, gene:60681-5APEX_IMS, mito protein domainIMSProtein import, sorting and homeostasis > Protein homeostasis > Chaperonesnone
SETD9SET ­domain ­containing ­9C5orf35, Q8NE22, gene:133383-5APEX_matrix, targetP signalMatrixnonenone
CYP11B1cytochrome ­P450 ­family ­11 ­subfamily ­B ­member ­1CPN1, CYP11B, FHI, P450C11, P15538, gene:1584-5manual, targetP signal, mito protein domainMIMMetabolism > Lipid metabolism > Cholesterol, bile acid, steroid synthesis;
Metabolism > Metals and cofactors > Heme-containing proteins
none
CSKMTcitrate ­synthase ­lysine ­methyltransferaseCS-KMT, METTL12, U99HG, A8MUP2, gene:751071-5literature, targetP signal, mito protein domainMatrixSignalingnone
MIGA1mitoguardin ­1FAM73A, Q8NAN2, gene:374986-5literatureMOMMitochondrial dynamics and surveillance > Fusioncerebrum, cerebellum, brainstem, spinalcord, smallintestine, testis
RPIAribose ­5-phosphate ­isomerase ­ARPI, RPIAD, P49247, gene:22934-5APEX_matrixMatrixMetabolism > Carbohydrate metabolismplacenta
BBC3BCL2 ­binding ­component ­3JFY-1, JFY1, PUMA, Q96PG8, gene:27113-6manual MOMMitochondrial dynamics and surveillance > Apoptosisnone
BADBCL2 ­associated ­agonist ­of ­cell ­deathBBC2, BCL2L8, Q92934, gene:572-6literature, mito protein domainMOMMitochondrial dynamics and surveillance > Apoptosisnone
BCL2BCL2 ­apoptosis ­regulatorBcl-2, PPP1R50, P10415, gene:596-6literature, mito protein domainMOMMitochondrial dynamics and surveillance > Apoptosisnone
BCL2A1BCL2 ­related ­protein ­A1ACC-1, ACC-2, ACC1, ACC2, BCL2L5, BFL1, GRS, HBPA1, Q16548, gene:597-6manual, mito protein domainMOMMitochondrial dynamics and surveillance > Apoptosisnone
BCL2L2BCL2 ­like ­2BCL-W, BCL2-L-2, BCLW, PPP1R51, Q92843, gene:599-6literature, mito protein domainMOMMitochondrial dynamics and surveillance > Apoptosisnone
CASP3caspase ­3CPP32, CPP32B, SCA-1, P42574, gene:836-6literature, mito protein domainIMSProtein import, sorting and homeostasis > Protein homeostasis > Proteases;
Mitochondrial dynamics and surveillance > Apoptosis
none
DMPKDM1 ­protein ­kinaseDM, DM1, DM1PK, DMK, MDPK, MT-PK, Q09013, gene:1760-6literature, mito protein domainMOMnonenone
DNA2DNA ­replication ­helicase/nuclease ­2DNA2L, hDNA2, P51530, gene:1763-6literature, mito protein domainMatrixMitochondrial central dogma > mtDNA maintenance > mtDNA replication;
Metabolism > Metals and cofactors > Fe-S-containing proteins
none
MT-ND6NADH ­dehydrogenase, ­subunit ­6 ­(complex ­I)MTND6, P03923, gene:4541-6literature, mito protein domainMIMOXPHOS > Complex I > CI subunits;
OXPHOS > OXPHOS subunits
none
PRKNparkin ­RBR ­E3 ­ubiquitin ­protein ­ligaseAR-JP, LPRS2, PARK2, PDJ, O60260, gene:5071-6manual, mito protein domainMOMMitochondrial dynamics and surveillance > Mitophagy;
Mitochondrial dynamics and surveillance > Autophagy
none
ALKBH1alkB ­homolog ­1, ­histone ­H2A ­dioxygenaseABH, ABH1, ALKBH, alkB, hABH, Q13686, gene:8846-6literature, APEX_matrix, mito protein domainMatrixMitochondrial central dogma > mtRNA metabolism > mtRNA granules;
Mitochondrial central dogma > mtRNA metabolism > mt-tRNA modifications
none
PICK1protein ­interacting ­with ­PRKCA ­1PICK, PRKCABP, Q9NRD5, gene:9463-6literature, mito protein domainunknownnonenone
BCL2L10BCL2 ­like ­10BCL-B, Boo, Diva, bcl2-L-10, Q9HD36, gene:10017-6manual, mito protein domainMOMMitochondrial dynamics and surveillance > Apoptosisnone
AKAP10A-kinase ­anchoring ­protein ­10AKAP-10, D-AKAP-2, D-AKAP2, PRKA10, O43572, gene:11216-6literature, mito protein domainMOMSignaling > cAMP-PKA signalingnone
STYXL1serine/threonine/tyrosine ­interacting ­like ­1DUSP24, MK-STYX, MKSTYX, Q9Y6J8, gene:51657-6literature, mito protein domainMatrixMitochondrial dynamics and surveillance > Apoptosisnone
NSUN2NOP2/Sun ­RNA ­methyltransferase ­2MISU, MRT5, SAKI, TRM4, Q08J23, gene:54888-6literature, mito protein domainMatrixMitochondrial central dogma > mtRNA metabolism > mt-tRNA modificationsnone
ADCY10adenylate ­cyclase ­10HCA2, HEL-S-7a, SAC, SACI, Sacy, hsAC, Q96PN6, gene:55811-6literature, mito protein domainMembraneSignaling > cAMP-PKA signalingnone
SPHK2sphingosine ­kinase ­2SK 2, SK-2, SPK 2, SPK-2, Q9NRA0, gene:56848-6literature, mito protein domainMIMMetabolism > Lipid metabolism > Phospholipid metabolism;
Mitochondrial dynamics and surveillance > Apoptosis
none
NSUN3NOP2/Sun ­RNA ­methyltransferase ­3MST077, MSTP077, Q9H649, gene:63899-6APEX_matrix, mito protein domainMatrixMitochondrial central dogma > mtRNA metabolism > mt-tRNA modificationsnone
PABPC5poly(A) ­binding ­protein ­cytoplasmic ­5PABP5, Q96DU9, gene:140886-6literature, mito protein domainMatrixnonenone
PRSS35serine ­protease ­35C6orf158, dJ223E3.1, Q8N3Z0, gene:167681-6GFP, mito protein domainunknownProtein import, sorting and homeostasis > Protein homeostasisnone
PRIMPOLprimase ­and ­DNA ­directed ­polymeraseCCDC111, MYP22, Primpol1, Q96LW4, gene:201973-6literature, mito protein domainMatrixMitochondrial central dogma > mtDNA maintenance > mtDNA repairnone
SERAC1serine ­active ­site ­containing ­1Q96JX3, gene:84947-6literature, APEX_IMSMOMMetabolism > Lipid metabolism > Phospholipid metabolismcerebrum, cerebellum, skeletalmuscle, stomach, testis
STX17syntaxin ­17P56962, gene:55014-6APEX_IMS, coexpressionMOMMitochondrial dynamics and surveillance > Fission;
Mitochondrial dynamics and surveillance > Organelle contact sites;
Mitochondrial dynamics and surveillance > Autophagy
testis
TRMT61BtRNA ­methyltransferase ­61BQ9BVS5, gene:55006-7APEX_matrix, mito protein domainMatrixMitochondrial central dogma > mtRNA metabolism > mt-tRNA modifications;
Mitochondrial central dogma > mtRNA metabolism > mt-rRNA modifications;
Mitochondrial central dogma > Translation > Mitochondrial ribosome assembly
none
PDE2Aphosphodiesterase ­2ACGS-PDE, PDE2A1, PED2A4, cGSPDE, O00408, gene:5138-7literatureMIMSignaling > cAMP-PKA signalingcerebrum, cerebellum, brainstem, largeintestine
PLGRKTplasminogen ­receptor ­with ­a ­C-terminal ­lysineAD025, C9orf46, MDS030, PLG-RKT, Plg-R(KT), Q9HBL7, gene:55848-7GFPMembranenonecerebrum, cerebellum, brainstem, spinalcord, skeletalmuscle, adipose, smallintestine, largeintestine, stomach, placenta, testis
ETFBKMTelectron ­transfer ­flavoprotein ­subunit ­beta ­lysine ­methyltransferaseC12orf72, ETFB-KMT, METTL20, Q8IXQ9, gene:254013-7literature, targetP signalMatrixMetabolism > Electron carriers > Q-linked reactions, othernone
RTL10retrotransposon ­Gag ­like ­10BOP, C22orf29, Q7L3V2, gene:79680-7manual, targetP signalMOMMitochondrial dynamics and surveillance > Apoptosisnone
BCL2L11BCL2 ­like ­11BAM, BIM, BOD, O43521, gene:10018-8manual MOMMitochondrial dynamics and surveillance > Apoptosisnone
SPIRE1spire ­type ­actin ­nucleation ­factor ­1Spir-1, Q08AE8, gene:56907-8literatureMOMMitochondrial dynamics and surveillance > Fission;
Mitochondrial dynamics and surveillance > Organelle contact sites
none
C2orf69chromosome ­2 ­open ­reading ­frame ­69Q8N8R5, gene:205327-8APEX_matrixMatrixnonenone
ARMCX2armadillo ­repeat ­containing ­X-linked ­2ALEX2, GASP9, Q7L311, gene:9823-10literatureMembranenonenone
ARMCX1armadillo ­repeat ­containing ­X-linked ­1ALEX1, GASP7, Q9P291, gene:51309-10literatureMOMMitochondrial dynamics and surveillance > Traffickingnone
POLBDNA ­polymerase ­betaP06746, gene:5423-10literatureMatrixMitochondrial central dogma > mtDNA maintenance > mtDNA repairnone
SNAP29synaptosome ­associated ­protein ­29CEDNIK, SNAP-29, O95721, gene:9342-10APEX_IMSIMSMitochondrial dynamics and surveillance > Autophagynone
ARMCX3armadillo ­repeat ­containing ­X-linked ­3ALEX3, GASP6, dJ545K15.2, Q9UH62, gene:51566-10literatureMOMMitochondrial dynamics and surveillance > Traffickingnone
ARMCX6armadillo ­repeat ­containing ­X-linked ­6GASP10, Q7L4S7, gene:54470-10literatureMembranenonenone
METTL4methyltransferase ­like ­4HsT661, Q8N3J2, gene:64863-10literatureMatrixMitochondrial central dogma > mtDNA maintenance > mtDNA modificationsnone
SPHKAPSPHK1 ­interactor, ­AKAP ­domain ­containingSKIP, Q2M3C7, gene:80309-10manual MIMSignaling > cAMP-PKA signalingnone
NAT8LN-acetyltransferase ­8 ­likeCML3, NACED, NAT8-LIKE, Q8N9F0, gene:339983-10literatureMembraneMetabolism > Amino acid metabolism > Glutamate metabolismnone
MCCD1mitochondrial ­coiled-coil ­domain ­1P59942, gene:401250-10manual unknownnonenone
PIGBOS1PIGB ­opposite ­strand ­1HP06981, A0A0B4J2F0, gene:101928527NAliteratureMOMMitochondrial dynamics and surveillance > Organelle contact sitesNA
HTD2hydroxyacyl-thioester ­dehydratase ­type ­2P86397, gene:109703458NAliteratureMatrixMetabolism > Lipid metabolism > Type II fatty acid synthesisNA
RP11_469A15.2RP11_469A15.20, gene:ENSG00000230623NAliteratureMIMNA