MitoPathways3.0: 149 hierarchical pathways

MitoCarta3.0 genes have been annotated into a hierarchy of 149 biological pathways based on literature curation. More complete information is available for download in file Human.MitoCarta3.0.xls and Mouse.MitoCarta3.0.xls. See also MitoCarta3.0 home for a description of the project.

MitoPathwayMitoPathways HierarchyHuman MitoCarta3.0 Genes
Mitochondrial central dogmaMitochondrial central dogmaAARS2, ALKBH1, ANGEL2, APEX1, ATAD3A, ATAD3B, AURKAIP1, C12orf65, CARS2, CDK5RAP1, CHCHD1, COA3, COX14, DAP3, DARS2, DDX28, DHX30, DNA2, DUS2, EARS2, ELAC2, ENDOG, ERAL1, EXD2, EXOG, FARS2, FASTK, FASTKD1, FASTKD2, FASTKD3, FASTKD5, GADD45GIP1, GARS1, GATB, GATC, GFM1, GFM2, GRSF1, GTPBP10, GTPBP3, GUF1, HARS2, HEMK1, HSD17B10, IARS2, KARS1, LACTB2, LARS2, LIG3, LRPPRC, MALSU1, MARS2, METAP1D, METTL15, METTL17, METTL4, METTL5, METTL8, MGME1, MIEF1, MPV17L2, MRM1, MRM2, MRM3, MRPL1, MRPL10, MRPL11, MRPL12, MRPL13, MRPL14, MRPL15, MRPL16, MRPL17, MRPL18, MRPL19, MRPL2, MRPL20, MRPL21, MRPL22, MRPL23, MRPL24, MRPL27, MRPL28, MRPL3, MRPL30, MRPL32, MRPL33, MRPL34, MRPL35, MRPL36, MRPL37, MRPL38, MRPL39, MRPL4, MRPL40, MRPL41, MRPL42, MRPL43, MRPL44, MRPL45, MRPL46, MRPL47, MRPL48, MRPL49, MRPL50, MRPL51, MRPL52, MRPL53, MRPL54, MRPL55, MRPL57, MRPL58, MRPL9, MRPS10, MRPS11, MRPS12, MRPS14, MRPS15, MRPS16, MRPS17, MRPS18A, MRPS18B, MRPS18C, MRPS2, MRPS21, MRPS22, MRPS23, MRPS24, MRPS25, MRPS26, MRPS27, MRPS28, MRPS30, MRPS31, MRPS33, MRPS34, MRPS35, MRPS36, MRPS5, MRPS6, MRPS7, MRPS9, MRRF, MTERF1, MTERF2, MTERF3, MTERF4, MTFMT, MTG1, MTG2, MTIF2, MTIF3, MTO1, MTPAP, MTRES1, MTRF1, MTRF1L, MUTYH, MYG1, NARS2, NGRN, NOA1, NSUN2, NSUN3, NSUN4, OGG1, OSGEPL1, OXA1L, PARS2, PDE12, PDF, PIF1, PNPT1, POLB, POLDIP2, POLG, POLG2, POLQ, POLRMT, PPA2, PRIMPOL, PRORP, PTCD1, PTCD2, PTCD3, PUS1, PUSL1, QRSL1, QTRT1, RARS2, RBFA, RCC1L, RECQL4, REXO2, RMND1, RNASEH1, RPUSD3, RPUSD4, SARS2, SLIRP, SSBP1, SUPV3L1, TACO1, TARS2, TBRG4, TEFM, TFAM, TFB1M, TFB2M, THG1L, TIMM21, TOP1MT, TOP3A, TRIT1, TRMT1, TRMT10C, TRMT2B, TRMT5, TRMT61B, TRMU, TRNT1, TRUB2, TSFM, TUFM, TWNK, UNG, VARS2, WARS2, YARS2, YBEY, YRDC
mtDNA maintenanceMitochondrial central dogma > mtDNA maintenanceAPEX1, ATAD3A, ATAD3B, DNA2, ENDOG, EXOG, LIG3, METTL4, MGME1, MTERF1, MTERF2, MUTYH, OGG1, PIF1, POLB, POLDIP2, POLG, POLG2, POLQ, POLRMT, PPA2, PRIMPOL, RECQL4, RNASEH1, SSBP1, TFAM, TFB2M, TOP1MT, TOP3A, TWNK, UNG
mtDNA replicationMitochondrial central dogma > mtDNA maintenance > mtDNA replicationDNA2, EXOG, LIG3, MGME1, POLG, POLG2, POLRMT, RNASEH1, SSBP1, TFAM, TFB2M, TOP1MT, TOP3A, TWNK
mtDNA nucleoidMitochondrial central dogma > mtDNA maintenance > mtDNA nucleoidATAD3A, ATAD3B, MTERF1, MTERF2, POLDIP2, POLG, POLG2, POLRMT, SSBP1, TFAM, TWNK
mtDNA repairMitochondrial central dogma > mtDNA maintenance > mtDNA repairAPEX1, EXOG, LIG3, OGG1, POLB, POLG, PRIMPOL, RECQL4, UNG
mtDNA modificationsMitochondrial central dogma > mtDNA maintenance > mtDNA modificationsMETTL4
mtDNA stability and decayMitochondrial central dogma > mtDNA maintenance > mtDNA stability and decayENDOG, EXOG, MGME1
mtRNA metabolismMitochondrial central dogma > mtRNA metabolismALKBH1, ANGEL2, CDK5RAP1, DDX28, DHX30, DUS2, ELAC2, ENDOG, ERAL1, FASTK, FASTKD1, FASTKD2, FASTKD3, FASTKD5, GRSF1, GTPBP3, HSD17B10, LACTB2, LRPPRC, METTL15, METTL17, METTL5, METTL8, MRM1, MRM2, MRM3, MRPL12, MRPL47, MRPS7, MRPS9, MTERF1, MTO1, MTPAP, MTRES1, MYG1, NGRN, NOA1, NSUN2, NSUN3, NSUN4, OSGEPL1, PDE12, PNPT1, POLRMT, PPA2, PRORP, PTCD1, PTCD2, PUS1, QTRT1, RCC1L, REXO2, RMND1, RNASEH1, RPUSD3, RPUSD4, SLIRP, SUPV3L1, TBRG4, TEFM, TFAM, TFB1M, TFB2M, THG1L, TOP1MT, TRIT1, TRMT1, TRMT10C, TRMT2B, TRMT5, TRMT61B, TRMU, TRNT1, TRUB2, YBEY, YRDC
TranscriptionMitochondrial central dogma > mtRNA metabolism > TranscriptionMRPL12, MTERF1, MTRES1, POLRMT, TEFM, TFAM, TFB2M, TOP1MT
mtRNA granulesMitochondrial central dogma > mtRNA metabolism > mtRNA granulesALKBH1, DDX28, DHX30, ERAL1, FASTK, FASTKD1, FASTKD2, FASTKD5, GRSF1, HSD17B10, MRM1, MRM2, MRPL47, MRPS7, MRPS9, MTPAP, NOA1, PRORP, PTCD1, PTCD2, PUS1, RMND1, RPUSD3, RPUSD4, SUPV3L1, TFB1M, TRMT10C, TRUB2
Polycistronic mtRNA processingMitochondrial central dogma > mtRNA metabolism > Polycistronic mtRNA processingELAC2, FASTK, FASTKD1, FASTKD2, FASTKD3, FASTKD5, GRSF1, HSD17B10, PRORP, PTCD2, TBRG4, TRMT10C, YBEY
mt-tRNA modificationsMitochondrial central dogma > mtRNA metabolism > mt-tRNA modificationsALKBH1, CDK5RAP1, DUS2, GTPBP3, HSD17B10, MTO1, NSUN2, NSUN3, OSGEPL1, PUS1, QTRT1, RPUSD4, THG1L, TRIT1, TRMT1, TRMT10C, TRMT2B, TRMT5, TRMT61B, TRMU, TRNT1, YRDC
mt-rRNA modificationsMitochondrial central dogma > mtRNA metabolism > mt-rRNA modificationsMETTL15, METTL17, METTL5, MRM1, MRM2, MRM3, MTPAP, MYG1, NGRN, NSUN4, RCC1L, RPUSD3, RPUSD4, TFB1M, TRIT1, TRMT2B, TRMT61B, TRUB2
mt-mRNA modificationsMitochondrial central dogma > mtRNA metabolism > mt-mRNA modificationsANGEL2, MTPAP, MYG1, PDE12
mtRNA stability and decayMitochondrial central dogma > mtRNA metabolism > mtRNA stability and decayENDOG, FASTK, FASTKD1, FASTKD2, FASTKD3, FASTKD5, GRSF1, LACTB2, LRPPRC, MTPAP, PDE12, PNPT1, REXO2, RNASEH1, SLIRP, SUPV3L1, TBRG4
TranslationMitochondrial central dogma > TranslationAARS2, AURKAIP1, C12orf65, CARS2, CHCHD1, COA3, COX14, DAP3, DARS2, DDX28, DHX30, EARS2, ERAL1, EXD2, FARS2, FASTKD2, GADD45GIP1, GARS1, GATB, GATC, GFM1, GFM2, GRSF1, GTPBP10, GUF1, HARS2, HEMK1, IARS2, KARS1, LARS2, LRPPRC, MALSU1, MARS2, METAP1D, METTL17, MIEF1, MPV17L2, MRM2, MRM3, MRPL1, MRPL10, MRPL11, MRPL12, MRPL13, MRPL14, MRPL15, MRPL16, MRPL17, MRPL18, MRPL19, MRPL2, MRPL20, MRPL21, MRPL22, MRPL23, MRPL24, MRPL27, MRPL28, MRPL3, MRPL30, MRPL32, MRPL33, MRPL34, MRPL35, MRPL36, MRPL37, MRPL38, MRPL39, MRPL4, MRPL40, MRPL41, MRPL42, MRPL43, MRPL44, MRPL45, MRPL46, MRPL47, MRPL48, MRPL49, MRPL50, MRPL51, MRPL52, MRPL53, MRPL54, MRPL55, MRPL57, MRPL58, MRPL9, MRPS10, MRPS11, MRPS12, MRPS14, MRPS15, MRPS16, MRPS17, MRPS18A, MRPS18B, MRPS18C, MRPS2, MRPS21, MRPS22, MRPS23, MRPS24, MRPS25, MRPS26, MRPS27, MRPS28, MRPS30, MRPS31, MRPS33, MRPS34, MRPS35, MRPS36, MRPS5, MRPS6, MRPS7, MRPS9, MRRF, MTERF3, MTERF4, MTFMT, MTG1, MTG2, MTIF2, MTIF3, MTRES1, MTRF1, MTRF1L, NARS2, NGRN, NOA1, NSUN4, OXA1L, PARS2, PDF, PPA2, PTCD3, PUSL1, QRSL1, RARS2, RBFA, RMND1, SARS2, SLIRP, TACO1, TARS2, TFB1M, TIMM21, TRMT61B, TSFM, TUFM, VARS2, WARS2, YARS2, YBEY
Mitochondrial ribosomeMitochondrial central dogma > Translation > Mitochondrial ribosomeAURKAIP1, CHCHD1, DAP3, GADD45GIP1, MRPL1, MRPL10, MRPL11, MRPL12, MRPL13, MRPL14, MRPL15, MRPL16, MRPL17, MRPL18, MRPL19, MRPL2, MRPL20, MRPL21, MRPL22, MRPL23, MRPL24, MRPL27, MRPL28, MRPL3, MRPL30, MRPL32, MRPL33, MRPL34, MRPL35, MRPL36, MRPL37, MRPL38, MRPL39, MRPL4, MRPL40, MRPL41, MRPL42, MRPL43, MRPL44, MRPL45, MRPL46, MRPL47, MRPL48, MRPL49, MRPL50, MRPL51, MRPL52, MRPL53, MRPL54, MRPL55, MRPL57, MRPL58, MRPL9, MRPS10, MRPS11, MRPS12, MRPS14, MRPS15, MRPS16, MRPS17, MRPS18A, MRPS18B, MRPS18C, MRPS2, MRPS21, MRPS22, MRPS23, MRPS24, MRPS25, MRPS26, MRPS27, MRPS28, MRPS30, MRPS31, MRPS33, MRPS34, MRPS35, MRPS36, MRPS5, MRPS6, MRPS7, MRPS9, PTCD3
Mitochondrial ribosome assemblyMitochondrial central dogma > Translation > Mitochondrial ribosome assemblyDDX28, DHX30, ERAL1, FASTKD2, GRSF1, GTPBP10, MALSU1, METTL17, MIEF1, MPV17L2, MRM2, MRM3, MTERF3, MTERF4, MTG1, MTG2, NGRN, NOA1, NSUN4, PTCD3, RBFA, TFB1M, TRMT61B, YBEY
Translation factorsMitochondrial central dogma > Translation > Translation factorsC12orf65, COA3, COX14, GFM1, GFM2, HEMK1, MRPL58, MRRF, MTIF2, MTIF3, MTRF1, MTRF1L, TACO1, TIMM21, TSFM, TUFM
mt-tRNA synthetasesMitochondrial central dogma > Translation > mt-tRNA synthetasesAARS2, CARS2, DARS2, EARS2, FARS2, GARS1, GATB, GATC, HARS2, IARS2, KARS1, LARS2, MARS2, NARS2, PARS2, QRSL1, RARS2, SARS2, TARS2, VARS2, WARS2, YARS2
fMet processingMitochondrial central dogma > Translation > fMet processingMETAP1D, MTFMT, PDF
Protein import, sorting and homeostasisProtein import, sorting and homeostasisAFG1L, AFG3L2, AGK, AIFM1, ATAD1, ATP23, CASP3, CASP8, CASP9, CHCHD4, CLPB, CLPP, CLPX, DNAJA3, DNAJC11, DNAJC15, DNAJC19, DNAJC4, DNLZ, FKBP10, GFER, GRPEL1, GRPEL2, HSPD1, HSPE1, HTRA2, IDE, IMMP1L, IMMP2L, LACTB, LAP3, LONP1, MAIP1, METAP1D, MIPEP, MTX1, MTX2, MUL1, NLN, NRDC, OMA1, OXA1L, PAM16, PARK7, PARL, PDF, PHB, PHB2, PITRM1, PMPCA, PMPCB, PRSS35, ROMO1, SAMM50, SPG7, STOML2, TIMM10, TIMM10B, TIMM13, TIMM17A, TIMM17B, TIMM21, TIMM22, TIMM23, TIMM29, TIMM44, TIMM50, TIMM8A, TIMM8B, TIMM9, TOMM20, TOMM20L, TOMM22, TOMM34, TOMM40, TOMM40L, TOMM5, TOMM6, TOMM7, TOMM70, TRAP1, UQCRC1, UQCRC2, USP30, XPNPEP3, YME1L1
Protein import and sortingProtein import, sorting and homeostasis > Protein import and sortingAGK, AIFM1, CHCHD4, DNAJC11, DNAJC15, DNAJC19, GFER, GRPEL1, GRPEL2, IMMP1L, IMMP2L, MIPEP, MTX1, MTX2, NRDC, OXA1L, PAM16, PMPCA, PMPCB, ROMO1, SAMM50, TIMM10, TIMM10B, TIMM13, TIMM17A, TIMM17B, TIMM21, TIMM22, TIMM23, TIMM29, TIMM44, TIMM50, TIMM8A, TIMM8B, TIMM9, TOMM20, TOMM20L, TOMM22, TOMM34, TOMM40, TOMM40L, TOMM5, TOMM6, TOMM7, TOMM70, UQCRC1, UQCRC2, XPNPEP3
TOMProtein import, sorting and homeostasis > Protein import and sorting > TOMTOMM20, TOMM22, TOMM34, TOMM40, TOMM40L, TOMM5, TOMM6, TOMM7, TOMM70
SAMProtein import, sorting and homeostasis > Protein import and sorting > SAMMTX1, MTX2, SAMM50
MIA40Protein import, sorting and homeostasis > Protein import and sorting > MIA40AIFM1, CHCHD4, GFER
TIM22 carrier pathwayProtein import, sorting and homeostasis > Protein import and sorting > TIM22 carrier pathwayAGK, TIMM22, TIMM29
TIM23 presequence pathwayProtein import, sorting and homeostasis > Protein import and sorting > TIM23 presequence pathwayROMO1, TIMM17A, TIMM17B, TIMM21, TIMM23, TIMM50
Import motorProtein import, sorting and homeostasis > Protein import and sorting > Import motorDNAJC15, DNAJC19, GRPEL1, GRPEL2, PAM16, TIMM44
OXAProtein import, sorting and homeostasis > Protein import and sorting > OXAOXA1L
Preprotein cleavageProtein import, sorting and homeostasis > Protein import and sorting > Preprotein cleavageIMMP1L, IMMP2L, MIPEP, PMPCA, PMPCB, UQCRC1, UQCRC2, XPNPEP3
Protein homeostasisProtein import, sorting and homeostasis > Protein homeostasisAFG1L, AFG3L2, ATAD1, ATP23, CASP3, CASP8, CASP9, CLPB, CLPP, CLPX, DNAJA3, DNAJC4, DNLZ, FKBP10, HSPD1, HSPE1, HTRA2, IDE, IMMP1L, IMMP2L, LACTB, LAP3, LONP1, MAIP1, METAP1D, MIPEP, MUL1, NLN, NRDC, OMA1, PARK7, PARL, PDF, PHB, PHB2, PITRM1, PMPCA, PMPCB, PRSS35, SPG7, STOML2, TIMM10, TIMM10B, TIMM13, TIMM8A, TIMM8B, TIMM9, TRAP1, USP30, XPNPEP3, YME1L1
ProteasesProtein import, sorting and homeostasis > Protein homeostasis > ProteasesAFG1L, AFG3L2, ATAD1, ATP23, CASP3, CASP9, CLPB, CLPP, CLPX, HTRA2, IDE, IMMP1L, IMMP2L, LACTB, LAP3, LONP1, METAP1D, MIPEP, NLN, OMA1, PARL, PITRM1, PMPCA, PMPCB, SPG7, USP30, XPNPEP3, YME1L1
ChaperonesProtein import, sorting and homeostasis > Protein homeostasis > ChaperonesDNAJA3, DNLZ, FKBP10, HSPD1, HSPE1, MAIP1, NRDC, PHB, PHB2, TIMM10, TIMM10B, TIMM13, TIMM8A, TIMM8B, TIMM9, TRAP1
OXPHOSOXPHOSACAD9, AIFM1, ATP5F1A, ATP5F1B, ATP5F1C, ATP5F1D, ATP5F1E, ATP5IF1, ATP5MC1, ATP5MC2, ATP5MC3, ATP5MD, ATP5ME, ATP5MF, ATP5MG, ATP5MPL, ATP5PB, ATP5PD, ATP5PF, ATP5PO, ATPAF1, ATPAF2, ATPSCKMT, BCS1L, CEP89, CMC1, CMC2, COA1, COA3, COA4, COA5, COA6, COA7, COA8, COX10, COX11, COX14, COX15, COX16, COX17, COX18, COX19, COX20, COX4I1, COX4I2, COX5A, COX5B, COX6A1, COX6A2, COX6B1, COX6B2, COX6C, COX7A1, COX7A2, COX7A2L, COX7B, COX7B2, COX7C, COX8A, COX8C, CYC1, CYCS, DMAC1, DMAC2, DMAC2L, ECSIT, FMC1, FOXRED1, HCCS, HIGD1A, HIGD2A, LYRM2, LYRM7, MT-ATP6, MT-ATP8, MT-CO1, MT-CO2, MT-CO3, MT-CYB, MT-ND1, MT-ND2, MT-ND3, MT-ND4, MT-ND4L, MT-ND5, MT-ND6, NDUFA1, NDUFA10, NDUFA11, NDUFA12, NDUFA13, NDUFA2, NDUFA3, NDUFA4, NDUFA5, NDUFA6, NDUFA7, NDUFA8, NDUFA9, NDUFAB1, NDUFAF1, NDUFAF2, NDUFAF3, NDUFAF4, NDUFAF5, NDUFAF6, NDUFAF7, NDUFAF8, NDUFB1, NDUFB10, NDUFB11, NDUFB2, NDUFB3, NDUFB4, NDUFB5, NDUFB6, NDUFB7, NDUFB8, NDUFB9, NDUFC1, NDUFC2, NDUFS1, NDUFS2, NDUFS3, NDUFS4, NDUFS5, NDUFS6, NDUFS7, NDUFS8, NDUFV1, NDUFV2, NDUFV3, NUBPL, PET100, PET117, PNKD, RAB5IF, SCO1, SCO2, SDHA, SDHAF1, SDHAF2, SDHAF3, SDHAF4, SDHB, SDHC, SDHD, SMIM20, SURF1, TACO1, TIMM21, TIMMDC1, TMEM126A, TMEM126B, TMEM177, TMEM186, TMEM70, TTC19, UQCC1, UQCC2, UQCC3, UQCR10, UQCR11, UQCRB, UQCRC1, UQCRC2, UQCRFS1, UQCRH, UQCRQ
OXPHOS
subunits
OXPHOS > OXPHOS subunitsATP5F1A, ATP5F1B, ATP5F1C, ATP5F1D, ATP5F1E, ATP5IF1, ATP5MC1, ATP5MC2, ATP5MC3, ATP5MD, ATP5ME, ATP5MF, ATP5MG, ATP5MPL, ATP5PB, ATP5PD, ATP5PF, ATP5PO, COX4I1, COX4I2, COX5A, COX5B, COX6A1, COX6A2, COX6B1, COX6B2, COX6C, COX7A1, COX7A2, COX7A2L, COX7B, COX7B2, COX7C, COX8A, COX8C, CYC1, CYCS, DMAC2L, HCCS, MT-ATP6, MT-ATP8, MT-CO1, MT-CO2, MT-CO3, MT-CYB, MT-ND1, MT-ND2, MT-ND3, MT-ND4, MT-ND4L, MT-ND5, MT-ND6, NDUFA1, NDUFA10, NDUFA11, NDUFA12, NDUFA13, NDUFA2, NDUFA3, NDUFA4, NDUFA5, NDUFA6, NDUFA7, NDUFA8, NDUFA9, NDUFAB1, NDUFB1, NDUFB10, NDUFB11, NDUFB2, NDUFB3, NDUFB4, NDUFB5, NDUFB6, NDUFB7, NDUFB8, NDUFB9, NDUFC1, NDUFC2, NDUFS1, NDUFS2, NDUFS3, NDUFS4, NDUFS5, NDUFS6, NDUFS7, NDUFS8, NDUFV1, NDUFV2, NDUFV3, SDHA, SDHB, SDHC, SDHD, UQCR10, UQCR11, UQCRB, UQCRC1, UQCRC2, UQCRFS1, UQCRH, UQCRQ
OXPHOS assembly factorsOXPHOS > OXPHOS assembly factorsACAD9, AIFM1, ATPAF1, ATPAF2, ATPSCKMT, BCS1L, CEP89, CMC1, CMC2, COA1, COA3, COA4, COA5, COA6, COA7, COA8, COX10, COX11, COX14, COX15, COX16, COX17, COX18, COX19, COX20, COX7A2L, DMAC1, DMAC2, ECSIT, FMC1, FOXRED1, HIGD1A, HIGD2A, LYRM2, LYRM7, NDUFAF1, NDUFAF2, NDUFAF3, NDUFAF4, NDUFAF5, NDUFAF6, NDUFAF7, NDUFAF8, NUBPL, PET100, PET117, PNKD, RAB5IF, SCO1, SCO2, SDHAF1, SDHAF2, SDHAF3, SDHAF4, SMIM20, SURF1, TACO1, TIMM21, TIMMDC1, TMEM126A, TMEM126B, TMEM177, TMEM186, TMEM70, TTC19, UQCC1, UQCC2, UQCC3
Complex IOXPHOS > Complex IACAD9, AIFM1, COA1, DMAC1, DMAC2, ECSIT, FOXRED1, LYRM2, MT-ND1, MT-ND2, MT-ND3, MT-ND4, MT-ND4L, MT-ND5, MT-ND6, NDUFA1, NDUFA10, NDUFA11, NDUFA12, NDUFA13, NDUFA2, NDUFA3, NDUFA5, NDUFA6, NDUFA7, NDUFA8, NDUFA9, NDUFAB1, NDUFAF1, NDUFAF2, NDUFAF3, NDUFAF4, NDUFAF5, NDUFAF6, NDUFAF7, NDUFAF8, NDUFB1, NDUFB10, NDUFB11, NDUFB2, NDUFB3, NDUFB4, NDUFB5, NDUFB6, NDUFB7, NDUFB8, NDUFB9, NDUFC1, NDUFC2, NDUFS1, NDUFS2, NDUFS3, NDUFS4, NDUFS5, NDUFS6, NDUFS7, NDUFS8, NDUFV1, NDUFV2, NDUFV3, NUBPL, TIMMDC1, TMEM126A, TMEM126B, TMEM186, TMEM70
CI subunitsOXPHOS > Complex I > CI subunitsMT-ND1, MT-ND2, MT-ND3, MT-ND4, MT-ND4L, MT-ND5, MT-ND6, NDUFA1, NDUFA10, NDUFA11, NDUFA12, NDUFA13, NDUFA2, NDUFA3, NDUFA5, NDUFA6, NDUFA7, NDUFA8, NDUFA9, NDUFAB1, NDUFB1, NDUFB10, NDUFB11, NDUFB2, NDUFB3, NDUFB4, NDUFB5, NDUFB6, NDUFB7, NDUFB8, NDUFB9, NDUFC1, NDUFC2, NDUFS1, NDUFS2, NDUFS3, NDUFS4, NDUFS5, NDUFS6, NDUFS7, NDUFS8, NDUFV1, NDUFV2, NDUFV3
CI assembly factorsOXPHOS > Complex I > CI assembly factorsACAD9, AIFM1, COA1, DMAC1, DMAC2, ECSIT, FOXRED1, LYRM2, NDUFAF1, NDUFAF2, NDUFAF3, NDUFAF4, NDUFAF5, NDUFAF6, NDUFAF7, NDUFAF8, NUBPL, TIMMDC1, TMEM126A, TMEM126B, TMEM186, TMEM70
Complex IIOXPHOS > Complex IISDHA, SDHAF1, SDHAF2, SDHAF3, SDHAF4, SDHB, SDHC, SDHD
CII subunitsOXPHOS > Complex II > CII subunitsSDHA, SDHB, SDHC, SDHD
CII assembly factorsOXPHOS > Complex II > CII assembly factorsSDHAF1, SDHAF2, SDHAF3, SDHAF4
Complex IIIOXPHOS > Complex IIIBCS1L, CYC1, LYRM7, MT-CYB, TTC19, UQCC1, UQCC2, UQCC3, UQCR10, UQCR11, UQCRB, UQCRC1, UQCRC2, UQCRFS1, UQCRH, UQCRQ
CIII subunitsOXPHOS > Complex III > CIII subunitsCYC1, MT-CYB, UQCR10, UQCR11, UQCRB, UQCRC1, UQCRC2, UQCRFS1, UQCRH, UQCRQ
CIII assembly factorsOXPHOS > Complex III > CIII assembly factorsBCS1L, LYRM7, TTC19, UQCC1, UQCC2, UQCC3
Complex IVOXPHOS > Complex IVCEP89, CMC1, CMC2, COA1, COA3, COA4, COA5, COA6, COA7, COA8, COX10, COX11, COX14, COX15, COX16, COX17, COX18, COX19, COX20, COX4I1, COX4I2, COX5A, COX5B, COX6A1, COX6A2, COX6B1, COX6B2, COX6C, COX7A1, COX7A2, COX7A2L, COX7B, COX7B2, COX7C, COX8A, COX8C, HIGD1A, MT-CO1, MT-CO2, MT-CO3, NDUFA4, PET100, PET117, PNKD, SCO1, SCO2, SMIM20, SURF1, TACO1, TIMM21, TMEM177
CIV subunitsOXPHOS > Complex IV > CIV subunitsCOX4I1, COX4I2, COX5A, COX5B, COX6A1, COX6A2, COX6B1, COX6B2, COX6C, COX7A1, COX7A2, COX7A2L, COX7B, COX7B2, COX7C, COX8A, COX8C, MT-CO1, MT-CO2, MT-CO3, NDUFA4
CIV assembly factorsOXPHOS > Complex IV > CIV assembly factorsCEP89, CMC1, CMC2, COA1, COA3, COA4, COA5, COA6, COA7, COA8, COX10, COX11, COX14, COX15, COX16, COX17, COX18, COX19, COX20, HIGD1A, PET100, PET117, PNKD, SCO1, SCO2, SMIM20, SURF1, TACO1, TIMM21, TMEM177
Complex VOXPHOS > Complex VATP5F1A, ATP5F1B, ATP5F1C, ATP5F1D, ATP5F1E, ATP5IF1, ATP5MC1, ATP5MC2, ATP5MC3, ATP5MD, ATP5ME, ATP5MF, ATP5MG, ATP5MPL, ATP5PB, ATP5PD, ATP5PF, ATP5PO, ATPAF1, ATPAF2, ATPSCKMT, DMAC2L, FMC1, MT-ATP6, MT-ATP8, TMEM70
CV subunitsOXPHOS > Complex V > CV subunitsATP5F1A, ATP5F1B, ATP5F1C, ATP5F1D, ATP5F1E, ATP5IF1, ATP5MC1, ATP5MC2, ATP5MC3, ATP5MD, ATP5ME, ATP5MF, ATP5MG, ATP5MPL, ATP5PB, ATP5PD, ATP5PF, ATP5PO, DMAC2L, MT-ATP6, MT-ATP8
CV assembly factorsOXPHOS > Complex V > CV assembly factorsATPAF1, ATPAF2, ATPSCKMT, FMC1, TMEM70
Respirasome assemblyOXPHOS > Respirasome assemblyCOX7A2L, HIGD1A, HIGD2A, RAB5IF
Cytochrome COXPHOS > Cytochrome CCYCS, HCCS
MetabolismMetabolismAADAT, AASS, ABAT, ABCB10, ABCB6, ABCB7, ABHD10, ABHD11, ACAA1, ACAA2, ACACA, ACACB, ACAD10, ACAD11, ACAD8, ACADL, ACADM, ACADS, ACADSB, ACADVL, ACAT1, ACLY, ACO2, ACOD1, ACOT11, ACOT13, ACOT2, ACOT7, ACOT9, ACP6, ACSF2, ACSF3, ACSL1, ACSL6, ACSM1, ACSM2A, ACSM2B, ACSM3, ACSM4, ACSM5, ACSS1, ACSS3, ADHFE1, AGK, AGMAT, AGPAT4, AGPAT5, AGXT, AGXT2, AIFM2, AIFM3, AK2, AK3, AK4, AKR1B10, AKR7A2, ALAS1, ALAS2, ALDH18A1, ALDH1B1, ALDH1L1, ALDH1L2, ALDH2, ALDH3A2, ALDH4A1, ALDH5A1, ALDH6A1, ALDH7A1, ALDH9A1, AMACR, AMT, ANTKMT, ARG2, AUH, BCAT2, BCKDHA, BCKDHB, BCKDK, BCO2, BDH1, BOLA1, BOLA3, BPHL, CA5A, CA5B, CAT, CBR3, CBR4, CHCHD7, CHDH, CHPT1, CISD1, CISD3, CKMT1A, CKMT1B, CKMT2, CLYBL, CMPK2, COASY, COMT, COQ10A, COQ10B, COQ2, COQ3, COQ4, COQ5, COQ6, COQ7, COQ8A, COQ8B, COQ9, COX10, COX11, COX15, COX17, COX19, CPOX, CPS1, CPT1A, CPT1B, CPT1C, CPT2, CRAT, CRLS1, CROT, CS, CYB5B, CYB5R3, CYC1, CYCS, CYP11A1, CYP11B1, CYP11B2, CYP24A1, CYP27A1, CYP27B1, D2HGDH, DBI, DBT, DCXR, DECR1, DGLUCY, DGUOK, DHODH, DHRS1, DHRS2, DHRS4, DHTKD1, DLAT, DLD, DLST, DMGDH, DNA2, DTYMK, DUT, ECH1, ECHDC1, ECHS1, ECI1, ECI2, EHHADH, EPHX2, ETFA, ETFB, ETFBKMT, ETFDH, ETFRF1, ETHE1, FABP1, FAHD1, FAM210B, FASN, FDPS, FDX1, FDX2, FDXR, FECH, FH, FHIT, FLAD1, FPGS, FTH1, FTMT, FXN, GATM, GCAT, GCDH, GCSH, GLDC, GLRX2, GLRX5, GLS, GLS2, GLUD1, GLUD2, GLYAT, GLYCTK, GOT2, GPAM, GPAT2, GPD2, GPT2, GPX1, GPX4, GRHPR, GRPEL1, GRPEL2, GSR, GSTK1, GSTZ1, GTPBP3, GUK1, HADH, HADHA, HADHB, HAGH, HAO2, HCCS, HIBADH, HIBCH, HINT1, HINT2, HMGCL, HMGCS2, HOGA1, HSCB, HSD17B10, HSD17B4, HSD17B8, HSPA9, HTD2, IBA57, IDH2, IDH3A, IDH3B, IDH3G, IDI1, ISCA1, ISCA2, ISCU, IVD, KMO, KYAT3, L2HGDH, LACTB, LDHAL6B, LDHB, LDHD, LIAS, LIPT1, LIPT2, LYPLA1, LYPLAL1, LYRM4, MAOA, MAOB, MCAT, MCCC1, MCCC2, MCEE, MDH2, ME2, ME3, MECR, MGST1, MGST3, MLYCD, MMAA, MMAB, MMADHC, MMUT, MOCS1, MPC1, MPC1L, MPC2, MPST, MRPS36, MSRA, MSRB2, MSRB3, MT-CO1, MT-CYB, MTARC1, MTARC2, MTFMT, MTHFD1L, MTHFD2, MTHFD2L, MTHFS, MTO1, NADK2, NAGS, NAT8L, NAXD, NAXE, NDUFAB1, NDUFS1, NDUFS2, NDUFS7, NDUFS8, NDUFV1, NDUFV2, NEU4, NFS1, NFU1, NIT1, NIT2, NME3, NME4, NME6, NMNAT3, NNT, NOCT, NT5DC2, NT5M, NTHL1, NUBPL, NUDT13, NUDT19, NUDT2, NUDT5, NUDT8, NUDT9, OAT, OGDH, OGDHL, OSBPL1A, OTC, OXCT1, OXCT2, OXR1, OXSM, PAICS, PANK2, PC, PCBD2, PCCA, PCCB, PCK2, PDHA1, PDHA2, PDHB, PDHX, PDK1, PDK2, PDK3, PDK4, PDP1, PDP2, PDPR, PDSS1, PDSS2, PGS1, PHYH, PISD, PLD6, PLPBP, PLSCR3, PNPLA8, PNPO, PPA2, PPM1K, PPOX, PRDX2, PRDX3, PRDX4, PRDX5, PRDX6, PRELID1, PRELID3A, PRELID3B, PRODH, PRODH2, PRXL2A, PTGES2, PTPMT1, PYCR1, PYCR2, QDPR, RDH13, RDH14, RFK, RPIA, RSAD1, SARDH, SCO1, SCO2, SCP2, SDHA, SDHB, SDHC, SDHD, SDSL, SELENOO, SERAC1, SFXN1, SFXN3, SFXN5, SHMT2, SIRT3, SIRT4, SIRT5, SLC25A1, SLC25A10, SLC25A11, SLC25A12, SLC25A13, SLC25A14, SLC25A15, SLC25A19, SLC25A20, SLC25A21, SLC25A23, SLC25A24, SLC25A25, SLC25A28, SLC25A29, SLC25A3, SLC25A30, SLC25A31, SLC25A32, SLC25A33, SLC25A36, SLC25A37, SLC25A38, SLC25A4, SLC25A41, SLC25A42, SLC25A44, SLC25A5, SLC25A51, SLC25A52, SLC25A6, SOD1, SOD2, SPHK2, SPR, SPTLC2, SQOR, STAR, STARD7, SUCLA2, SUCLG1, SUCLG2, SUGCT, SUOX, TAMM41, TAZ, THEM4, THEM5, TK2, TMLHE, TRIAP1, TSPO, TST, TSTD1, TXN2, TXNRD1, TXNRD2, UQCRFS1, ZADH2
Carbohydrate metabolismMetabolism > Carbohydrate metabolismABHD11, ACAA2, ACAT1, ACLY, ACO2, ACOD1, AUH, BDH1, CLYBL, CS, D2HGDH, DCXR, DLAT, DLD, DLST, FAHD1, FH, GLYCTK, GOT2, GPD2, HADHB, HMGCL, HMGCS2, IDH2, IDH3A, IDH3B, IDH3G, L2HGDH, LDHAL6B, LDHD, MCEE, MDH2, ME2, ME3, MMUT, MPC1, MPC1L, MPC2, MRPS36, NEU4, OGDH, OGDHL, OXCT1, OXCT2, PC, PCCA, PCCB, PCK2, PDHA1, PDHA2, PDHB, PDHX, PDK1, PDK2, PDK3, PDK4, PDP1, PDP2, PDPR, RPIA, SDHA, SDHB, SDHC, SDHD, SFXN5, SLC25A1, SLC25A10, SLC25A11, SLC25A12, SLC25A13, SUCLA2, SUCLG1, SUCLG2
GluconeogenesisMetabolism > Carbohydrate metabolism > GluconeogenesisMDH2, MPC1, MPC1L, MPC2, PC, PCK2, SLC25A1, SLC25A10, SLC25A11
Malate-aspartate shuttleMetabolism > Carbohydrate metabolism > Malate-aspartate shuttleGOT2, MDH2, SLC25A10, SLC25A11, SLC25A12, SLC25A13
Glycerol phosphate shuttleMetabolism > Carbohydrate metabolism > Glycerol phosphate shuttleGPD2
Pyruvate metabolismMetabolism > Carbohydrate metabolism > Pyruvate metabolismDLAT, DLD, MPC1, MPC1L, MPC2, PDHA1, PDHA2, PDHB, PDHX, PDK1, PDK2, PDK3, PDK4, PDP1, PDP2, PDPR
TCA cycleMetabolism > Carbohydrate metabolism > TCA cycleACO2, CS, DLD, DLST, FH, IDH2, IDH3A, IDH3B, IDH3G, MDH2, MRPS36, OGDH, OGDHL, SDHA, SDHB, SDHC, SDHD, SUCLA2, SUCLG1, SUCLG2
TCA-associatedMetabolism > Carbohydrate metabolism > TCA-associatedABHD11, ACLY, D2HGDH, FAHD1, L2HGDH, ME2, ME3, MPC1, MPC1L, MPC2, PC, PCK2, SFXN5, SLC25A1, SLC25A11
Itaconate metabolismMetabolism > Carbohydrate metabolism > Itaconate metabolismACOD1, AUH, CLYBL, SUCLA2, SUCLG1, SUCLG2
Ketone metabolismMetabolism > Carbohydrate metabolism > Ketone metabolismACAA2, ACAT1, BDH1, HADHB, HMGCL, HMGCS2, OXCT1, OXCT2
Propanoate metabolismMetabolism > Carbohydrate metabolism > Propanoate metabolismMCEE, MMUT, PCCA, PCCB
Lipid metabolismMetabolism > Lipid metabolismACAA1, ACAA2, ACACA, ACACB, ACAD10, ACAD11, ACADL, ACADM, ACADS, ACADSB, ACADVL, ACAT1, ACOT11, ACOT13, ACOT2, ACOT7, ACOT9, ACP6, ACSF2, ACSF3, ACSL1, ACSL6, ACSM1, ACSM2A, ACSM2B, ACSM3, ACSM4, ACSM5, ACSS1, ACSS3, AGK, AGPAT4, AGPAT5, AMACR, CBR4, CHPT1, CPT1A, CPT1B, CPT1C, CPT2, CRAT, CRLS1, CROT, CYB5R3, CYP11A1, CYP11B1, CYP11B2, CYP27A1, DBI, DECR1, DHRS1, ECH1, ECHDC1, ECHS1, ECI1, ECI2, EHHADH, EPHX2, ETFA, ETFB, ETFDH, FABP1, FASN, FDPS, FDX1, FDXR, GCSH, GPAM, GPAT2, HADH, HADHA, HADHB, HAO2, HINT2, HSD17B10, HSD17B4, HSD17B8, HTD2, IDI1, LACTB, LIAS, LIPT1, LIPT2, LYPLA1, LYPLAL1, MCAT, MCEE, MECR, MGST3, MLYCD, MMUT, NDUFAB1, OSBPL1A, OXSM, PCCA, PCCB, PGS1, PHYH, PISD, PLD6, PLSCR3, PNPLA8, PRDX6, PRELID1, PRELID3A, PRELID3B, PTGES2, PTPMT1, SCP2, SERAC1, SLC25A1, SLC25A20, SPHK2, SPTLC2, STAR, STARD7, TAMM41, TAZ, THEM4, THEM5, TRIAP1, TSPO, ZADH2
Fatty acid oxidationMetabolism > Lipid metabolism > Fatty acid oxidationACAA2, ACACB, ACAD10, ACAD11, ACADL, ACADM, ACADS, ACADSB, ACADVL, ACAT1, ACOT11, ACSF2, ACSL1, ACSL6, ACSM1, ACSM2A, ACSM2B, ACSM3, ACSM4, ACSM5, ACSS1, AMACR, CPT1A, CPT1B, CPT1C, CPT2, CRAT, CROT, DECR1, ECHS1, ECI1, ECI2, ETFA, ETFB, ETFDH, HADH, HADHA, HADHB, HSD17B10, MCEE, MMUT, PCCA, PCCB, SLC25A20
Type II fatty acid synthesisMetabolism > Lipid metabolism > Type II fatty acid synthesisACACA, ACSF3, CBR4, HSD17B8, HTD2, MCAT, MECR, NDUFAB1, OXSM
Lipoate insertionMetabolism > Lipid metabolism > Lipoate insertionGCSH, LIAS, LIPT1, LIPT2
Cardiolipin synthesisMetabolism > Lipid metabolism > Cardiolipin synthesisAGPAT4, AGPAT5, CRLS1, GPAM, GPAT2, HADHA, PGS1, PLD6, PLSCR3, PNPLA8, PRELID1, PRELID3A, PTPMT1, TAMM41, TAZ, TRIAP1
Cholesterol, bile acid, steroid synthesisMetabolism > Lipid metabolism > Cholesterol, bile acid, steroid synthesisCYP11A1, CYP11B1, CYP11B2, CYP27A1, DHRS1, FDX1, FDXR, HINT2, HSD17B10, HSD17B8, OSBPL1A, SCP2, STAR, TSPO
Cholesterol-associatedMetabolism > Lipid metabolism > Cholesterol-associatedFDPS, IDI1
Phospholipid metabolismMetabolism > Lipid metabolism > Phospholipid metabolismACP6, AGK, AGPAT4, AGPAT5, CHPT1, GPAM, GPAT2, LACTB, OSBPL1A, PGS1, PISD, PNPLA8, PRDX6, PRELID1, PRELID3A, PRELID3B, PTPMT1, SERAC1, SPHK2, SPTLC2, STARD7, TAMM41, TRIAP1
Eicosanoid metabolismMetabolism > Lipid metabolism > Eicosanoid metabolismMGST3, PTGES2, ZADH2
Amino acid metabolismMetabolism > Amino acid metabolismAADAT, AASS, ABAT, ACAA2, ACAD8, ACADSB, ACAT1, ADHFE1, AGMAT, AGXT, AGXT2, AKR7A2, ALDH18A1, ALDH4A1, ALDH5A1, ALDH6A1, ALDH7A1, ALDH9A1, AMT, ARG2, AUH, BCAT2, BCKDHA, BCKDHB, BCKDK, CA5A, CA5B, CHDH, COMT, CPS1, DBT, DGLUCY, DHTKD1, DLD, DLST, DMGDH, ECHS1, ETFA, ETFB, ETFDH, GCAT, GCDH, GCSH, GLDC, GLS, GLS2, GLUD1, GLUD2, GLYAT, GOT2, GPT2, GRHPR, HADH, HADHA, HADHB, HIBADH, HIBCH, HMGCL, HOGA1, HSD17B10, IVD, KMO, KYAT3, LDHB, MAOA, MAOB, MCCC1, MCCC2, NAGS, NAT8L, OAT, OTC, PPM1K, PRODH, PRODH2, PYCR1, PYCR2, SARDH, SDSL, SFXN1, SFXN3, SHMT2, SLC25A12, SLC25A13, SLC25A15, SLC25A21, SLC25A29, SLC25A38, SLC25A44, SUGCT
Branched-chain amino acid metabolismMetabolism > Amino acid metabolism > Branched-chain amino acid metabolismACAD8, ACADSB, ACAT1, ALDH6A1, AUH, BCAT2, BCKDHA, BCKDHB, BCKDK, DBT, DLD, ECHS1, ETFA, ETFB, ETFDH, HADHA, HIBADH, HIBCH, HMGCL, HSD17B10, IVD, MCCC1, MCCC2, PPM1K, SLC25A44
Branched-chain amino acid dehydrogenase complexMetabolism > Amino acid metabolism > Branched-chain amino acid dehydrogenase complexBCKDHA, BCKDHB, BCKDK, DBT, DLD, PPM1K
Lysine metabolismMetabolism > Amino acid metabolism > Lysine metabolismAADAT, AASS, ACAA2, ACAT1, ALDH7A1, DHTKD1, DLD, DLST, ECHS1, ETFA, ETFB, ETFDH, GCDH, HADH, HADHB, SLC25A29
Serine metabolismMetabolism > Amino acid metabolism > Serine metabolismAGXT, SDSL, SFXN1, SFXN3, SHMT2
Glycine metabolismMetabolism > Amino acid metabolism > Glycine metabolismALDH7A1, AMT, CHDH, DLD, DMGDH, ETFA, ETFB, ETFDH, GCAT, GCSH, GLDC, GLYAT, SARDH, SHMT2, SLC25A38
Glycine cleavage systemMetabolism > Amino acid metabolism > Glycine cleavage systemAMT, DLD, GCSH, GLDC
Glutamate metabolismMetabolism > Amino acid metabolism > Glutamate metabolismGLS, GLS2, GLUD1, GLUD2, GOT2, GPT2, NAT8L, SLC25A12
Proline metabolismMetabolism > Amino acid metabolism > Proline metabolismALDH18A1, ALDH4A1, OAT, PRODH, PYCR1, PYCR2
Glyoxylate metabolismMetabolism > Amino acid metabolism > Glyoxylate metabolismAGXT, AGXT2, ALDH4A1, GOT2, GRHPR, HOGA1, LDHB, PRODH2
GABA metabolismMetabolism > Amino acid metabolism > GABA metabolismABAT, ADHFE1, AKR7A2, ALDH5A1, ALDH9A1, GLUD1, GLUD2
Catechol metabolismMetabolism > Amino acid metabolism > Catechol metabolismCOMT, MAOA, MAOB
Kynurenine metabolismMetabolism > Amino acid metabolism > Kynurenine metabolismGOT2, KMO, KYAT3
Urea cycleMetabolism > Amino acid metabolism > Urea cycleCA5A, CA5B, CPS1, NAGS, OTC, SLC25A13, SLC25A15
Nucleotide metabolismMetabolism > Nucleotide metabolismAK2, AK3, AK4, ANTKMT, CKMT1A, CKMT1B, CKMT2, CMPK2, DGUOK, DHODH, DTYMK, DUT, FHIT, GATM, GUK1, HINT1, NME3, NME4, NME6, NT5DC2, NT5M, NUDT2, NUDT5, NUDT9, PAICS, PPA2, SLC25A23, SLC25A24, SLC25A25, SLC25A31, SLC25A33, SLC25A36, SLC25A4, SLC25A41, SLC25A5, SLC25A6, SUCLA2, SUCLG1, SUCLG2, TK2
Nucleotide importMetabolism > Nucleotide metabolism > Nucleotide importSLC25A23, SLC25A24, SLC25A25, SLC25A31, SLC25A33, SLC25A36, SLC25A4, SLC25A41, SLC25A5, SLC25A6
Nucleotide synthesis and processingMetabolism > Nucleotide metabolism > Nucleotide synthesis and processingAK2, AK3, AK4, CMPK2, DGUOK, DHODH, DTYMK, DUT, FHIT, GUK1, HINT1, NME3, NME4, NME6, NT5DC2, NT5M, NUDT2, NUDT5, NUDT9, PAICS, SUCLA2, SUCLG1, SUCLG2, TK2
Creatine metabolismMetabolism > Nucleotide metabolism > Creatine metabolismCKMT1A, CKMT1B, CKMT2, GATM
Metals and cofactorsMetabolism > Metals and cofactorsABAT, ABCB10, ABCB6, ABCB7, ACO2, AIFM3, ALAS1, ALAS2, BOLA1, BOLA3, CHCHD7, CISD1, CISD3, COASY, COQ10A, COQ10B, COQ2, COQ3, COQ4, COQ5, COQ6, COQ7, COQ8A, COQ8B, COQ9, COX10, COX11, COX15, COX17, COX19, CPOX, CPT1A, CPT1B, CPT1C, CPT2, CYB5B, CYC1, CYCS, CYP11A1, CYP11B1, CYP11B2, CYP24A1, CYP27A1, CYP27B1, DNA2, ETFDH, FABP1, FAM210B, FDX1, FDX2, FDXR, FECH, FTH1, FTMT, FXN, GLRX2, GLRX5, GRPEL1, GRPEL2, HCCS, HSCB, HSPA9, IBA57, ISCA1, ISCA2, ISCU, KMO, KYAT3, LIAS, LYRM4, MOCS1, MT-CO1, MT-CYB, MTARC1, MTARC2, NADK2, NAXD, NAXE, NDUFAB1, NDUFS1, NDUFS2, NDUFS7, NDUFS8, NDUFV1, NDUFV2, NFS1, NFU1, NMNAT3, NNT, NOCT, NTHL1, NUBPL, NUDT13, NUDT19, NUDT8, PANK2, PCBD2, PDSS1, PDSS2, PPOX, QDPR, RSAD1, SCO1, SCO2, SDHB, SDHC, SDHD, SIRT3, SIRT4, SIRT5, SLC25A20, SLC25A28, SLC25A3, SLC25A37, SLC25A38, SLC25A42, SLC25A51, SLC25A52, SPR, SUOX, TMLHE, UQCRFS1
Carnitine synthesis and transportMetabolism > Metals and cofactors > Carnitine synthesis and transportCPT1A, CPT1B, CPT1C, CPT2, SLC25A20, TMLHE
Carnitine shuttleMetabolism > Metals and cofactors > Carnitine shuttleCPT1A, CPT1B, CPT1C, CPT2, SLC25A20
Coenzyme A metabolismMetabolism > Metals and cofactors > Coenzyme A metabolismCOASY, NUDT19, NUDT8, PANK2, SLC25A42
Coenzyme Q metabolismMetabolism > Metals and cofactors > Coenzyme Q metabolismCOQ10A, COQ10B, COQ2, COQ3, COQ4, COQ5, COQ6, COQ7, COQ8A, COQ8B, COQ9, PDSS1, PDSS2
Copper metabolismMetabolism > Metals and cofactors > Copper metabolismCHCHD7, COX11, COX17, COX19, SCO1, SCO2, SLC25A3
Iron homeostasisMetabolism > Metals and cofactors > Iron homeostasisFAM210B, FTH1, FTMT, SLC25A28, SLC25A37
Heme synthesis and processingMetabolism > Metals and cofactors > Heme synthesis and processingABCB10, ABCB6, ABCB7, ALAS1, ALAS2, COX10, COX15, CPOX, FABP1, FDX1, FDX2, FDXR, FECH, HCCS, PPOX, SLC25A38
Fe-S cluster biosynthesisMetabolism > Metals and cofactors > Fe-S cluster biosynthesisABCB7, BOLA1, BOLA3, CISD1, CISD3, FDX1, FDX2, FDXR, FXN, GLRX5, GRPEL1, GRPEL2, HSCB, HSPA9, IBA57, ISCA1, ISCA2, ISCU, LYRM4, NDUFAB1, NFS1, NFU1, NUBPL
Molybdenum cofactor synthesis and proteinsMetabolism > Metals and cofactors > Molybdenum cofactor synthesis and proteinsABCB7, MOCS1, MTARC1, MTARC2, SUOX
NAD biosynthesis and metabolismMetabolism > Metals and cofactors > NAD biosynthesis and metabolismKMO, KYAT3, NADK2, NAXD, NAXE, NMNAT3, NNT, NOCT, NUDT13, SIRT3, SIRT4, SIRT5, SLC25A51, SLC25A52
Tetrahydobiopterin synthesisMetabolism > Metals and cofactors > Tetrahydobiopterin synthesisPCBD2, QDPR, SPR
Fe-S-containing proteinsMetabolism > Metals and cofactors > Fe-S-containing proteinsABAT, ACO2, AIFM3, BOLA1, BOLA3, CISD1, CISD3, DNA2, ETFDH, FDX1, FDX2, FECH, GLRX2, GLRX5, ISCA1, ISCA2, ISCU, LIAS, NDUFS1, NDUFS2, NDUFS7, NDUFS8, NDUFV1, NDUFV2, NFS1, NFU1, NTHL1, NUBPL, RSAD1, SDHB, UQCRFS1
Heme-containing proteinsMetabolism > Metals and cofactors > Heme-containing proteinsCYB5B, CYC1, CYCS, CYP11A1, CYP11B1, CYP11B2, CYP24A1, CYP27A1, CYP27B1, MT-CO1, MT-CYB, SDHC, SDHD
Vitamin metabolismMetabolism > Vitamin metabolismACACA, ACACB, AKR1B10, ALDH1L1, ALDH1L2, ALDH7A1, AMT, BCO2, CHDH, CYP24A1, CYP27B1, DHRS4, DMGDH, ETFA, ETFB, ETFDH, FDX1, FDXR, FLAD1, FPGS, GLRX2, GTPBP3, MCCC1, MMAA, MMAB, MMADHC, MMUT, MTFMT, MTHFD1L, MTHFD2, MTHFD2L, MTHFS, MTO1, PC, PCCA, PLPBP, PNPO, QDPR, RDH13, RDH14, RFK, SARDH, SFXN1, SFXN3, SHMT2, SLC25A19, SLC25A32, SLC25A38, TXN2
Choline and betaine metabolismMetabolism > Vitamin metabolism > Choline and betaine metabolismALDH7A1, CHDH, DMGDH, ETFA, ETFB, ETFDH, SARDH
Biotin utilizing proteinsMetabolism > Vitamin metabolism > Biotin utilizing proteinsACACA, ACACB, MCCC1, PC, PCCA
Vitamin A metabolismMetabolism > Vitamin metabolism > Vitamin A metabolismAKR1B10, BCO2, DHRS4, RDH13, RDH14
Vitamin B1 metabolismMetabolism > Vitamin metabolism > Vitamin B1 metabolismSLC25A19
Vitamin B2 metabolismMetabolism > Vitamin metabolism > Vitamin B2 metabolismFLAD1, RFK, SLC25A32
Vitamin B6 metabolismMetabolism > Vitamin metabolism > Vitamin B6 metabolismPLPBP, PNPO
Vitamin B12 metabolismMetabolism > Vitamin metabolism > Vitamin B12 metabolismMMAA, MMAB, MMADHC, MMUT
Vitamin C metabolismMetabolism > Vitamin metabolism > Vitamin C metabolismGLRX2, TXN2
Vitamin D metabolismMetabolism > Vitamin metabolism > Vitamin D metabolismCYP24A1, CYP27B1, FDX1, FDXR
Folate and 1-C metabolismMetabolism > Vitamin metabolism > Folate and 1-C metabolismALDH1L1, ALDH1L2, ALDH7A1, AMT, CHDH, DMGDH, FPGS, GTPBP3, MTFMT, MTHFD1L, MTHFD2, MTHFD2L, MTHFS, MTO1, QDPR, SARDH, SFXN1, SFXN3, SHMT2, SLC25A32, SLC25A38
DetoxificationMetabolism > DetoxificationABHD10, ACSM2A, ACSM5, AIFM2, AKR1B10, AKR7A2, ALDH1B1, ALDH2, ALDH3A2, ALDH7A1, ALDH9A1, BPHL, CAT, CBR3, CYB5B, CYB5R3, DHRS2, EPHX2, GLRX2, GLYAT, GPX1, GPX4, GSR, GSTK1, GSTZ1, HAGH, MAOA, MAOB, MGST1, MPST, MSRA, MSRB2, MSRB3, MTARC1, MTARC2, NIT1, NIT2, OXR1, PRDX2, PRDX3, PRDX4, PRDX5, PRDX6, PRXL2A, SELENOO, SOD1, SOD2, TST, TXN2, TXNRD1, TXNRD2
Xenobiotic metabolismMetabolism > Detoxification > Xenobiotic metabolismABHD10, ACSM2A, ACSM5, AKR1B10, AKR7A2, ALDH1B1, ALDH2, BPHL, CBR3, CYB5B, CYB5R3, DHRS2, EPHX2, GLYAT, GSTK1, GSTZ1, MAOA, MAOB, MGST1, MPST, MTARC1, MTARC2, TST
ROS and glutathione metabolismMetabolism > Detoxification > ROS and glutathione metabolismAIFM2, ALDH3A2, ALDH7A1, ALDH9A1, CAT, GLRX2, GPX1, GPX4, GSR, HAGH, MGST1, MSRA, MSRB2, MSRB3, NIT1, OXR1, PRDX2, PRDX3, PRDX4, PRDX5, PRDX6, PRXL2A, SOD1, SOD2, TXN2, TXNRD1, TXNRD2
Amidoxime reducing complexMetabolism > Detoxification > Amidoxime reducing complexCYB5B, CYB5R3, MTARC1, MTARC2
SelenoproteinsMetabolism > Detoxification > SelenoproteinsGPX1, GPX4, SELENOO, TXNRD1, TXNRD2
Electron carriersMetabolism > Electron carriersCHDH, CYB5B, CYC1, CYCS, DHODH, ETFA, ETFB, ETFBKMT, ETFDH, ETFRF1, GPD2, MT-CYB, PRODH, PRODH2, SQOR
CytochromesMetabolism > Electron carriers > CytochromesCYB5B, CYC1, CYCS, MT-CYB
Q-linked reactions, otherMetabolism > Electron carriers > Q-linked reactions, otherCHDH, DHODH, ETFA, ETFB, ETFBKMT, ETFDH, ETFRF1, GPD2, PRODH, PRODH2, SQOR
Sulfur metabolismMetabolism > Sulfur metabolismETHE1, GOT2, MPST, MSRA, MSRB2, SLC25A14, SLC25A30, SQOR, SUOX, TST, TSTD1
Small molecule transportSmall molecule transportABCA9, ABCB10, ABCB6, ABCB7, ABCB8, ABCD1, ABCD2, ABCD3, CCDC51, LETMD1, MCU, MCUB, MICU1, MICU2, MICU3, MPC1, MPC1L, MPC2, MPV17, MPV17L, MRS2, MTCH1, MTCH2, ROMO1, SFXN1, SFXN2, SFXN3, SFXN4, SFXN5, SLC25A1, SLC25A10, SLC25A11, SLC25A12, SLC25A13, SLC25A14, SLC25A15, SLC25A16, SLC25A18, SLC25A19, SLC25A20, SLC25A21, SLC25A22, SLC25A23, SLC25A24, SLC25A25, SLC25A26, SLC25A27, SLC25A28, SLC25A29, SLC25A3, SLC25A30, SLC25A31, SLC25A32, SLC25A33, SLC25A34, SLC25A35, SLC25A36, SLC25A37, SLC25A38, SLC25A39, SLC25A4, SLC25A40, SLC25A41, SLC25A42, SLC25A43, SLC25A44, SLC25A45, SLC25A46, SLC25A47, SLC25A48, SLC25A5, SLC25A51, SLC25A52, SLC25A53, SLC25A6, SLC30A9, SLC8B1, SMDT1, STARD7, UCP1, UCP2, UCP3, VDAC1, VDAC2, VDAC3
SLC25A familySmall molecule transport > SLC25A familyMTCH1, MTCH2, SLC25A1, SLC25A10, SLC25A11, SLC25A12, SLC25A13, SLC25A14, SLC25A15, SLC25A16, SLC25A18, SLC25A19, SLC25A20, SLC25A21, SLC25A22, SLC25A23, SLC25A24, SLC25A25, SLC25A26, SLC25A27, SLC25A28, SLC25A29, SLC25A3, SLC25A30, SLC25A31, SLC25A32, SLC25A33, SLC25A34, SLC25A35, SLC25A36, SLC25A37, SLC25A38, SLC25A39, SLC25A4, SLC25A40, SLC25A41, SLC25A42, SLC25A43, SLC25A44, SLC25A45, SLC25A46, SLC25A47, SLC25A48, SLC25A5, SLC25A51, SLC25A52, SLC25A53, SLC25A6, UCP1, UCP2, UCP3
ABC transportersSmall molecule transport > ABC transportersABCA9, ABCB10, ABCB6, ABCB7, ABCB8, ABCD1, ABCD2, ABCD3
SideroflexinsSmall molecule transport > SideroflexinsSFXN1, SFXN2, SFXN3, SFXN4, SFXN5
Calcium uniporterSmall molecule transport > Calcium uniporterMCU, MCUB, MICU1, MICU2, MICU3, SMDT1
SignalingSignalingACOD1, ADCY10, AKAP1, AKAP10, BLOC1S1, C1QBP, CSKMT, DELE1, EFHD1, FUNDC2, IFI27, LETM1, LYPLA1, MACROD1, MAVS, MCRIP2, MCU, MCUB, MICU1, MICU2, MICU3, MRS2, MUL1, NLRX1, OCIAD2, PDE2A, PINK1, PPIF, PPTC7, PRKACA, RHOT1, RHOT2, SIRT3, SLC25A12, SLC25A13, SLC25A23, SLC25A24, SLC25A25, SLC25A3, SLC25A31, SLC25A4, SLC25A5, SLC25A6, SLC8B1, SMDT1, SPHKAP, VDAC1, VDAC3
Calcium homeostasisSignaling > Calcium homeostasisEFHD1, LETM1, MCU, MCUB, MICU1, MICU2, MICU3, PPIF, RHOT1, RHOT2, SLC25A12, SLC25A13, SLC25A23, SLC25A24, SLC25A25, SLC25A3, SLC25A31, SLC25A4, SLC25A5, SLC25A6, SLC8B1, SMDT1, VDAC1, VDAC3
Calcium cycleSignaling > Calcium homeostasis > Calcium cycleLETM1, MCU, MCUB, MICU1, MICU2, MICU3, SLC25A3, SLC8B1, SMDT1
Mitochondrial permeability transition poreSignaling > Calcium homeostasis > Mitochondrial permeability transition porePPIF, SLC25A31, SLC25A4, SLC25A5, SLC25A6, VDAC1, VDAC3
EF hand proteinsSignaling > Calcium homeostasis > EF hand proteinsEFHD1, MICU1, MICU2, MICU3, RHOT1, RHOT2, SLC25A12, SLC25A13, SLC25A23, SLC25A24, SLC25A25
Immune responseSignaling > Immune responseACOD1, C1QBP, IFI27, MAVS, NLRX1
cAMP-PKA signalingSignaling > cAMP-PKA signalingADCY10, AKAP1, AKAP10, PDE2A, PRKACA, SPHKAP
Mitochondrial dynamics and surveillanceMitochondrial dynamics and surveillanceAHCYL1, AIFM1, AIFM2, AIFM3, APOO, APOOL, ARL2, ARMC10, ARMCX1, ARMCX3, ATAD3A, ATAD3B, ATP5MD, ATP5ME, ATP5MG, BAD, BAK1, BAX, BBC3, BCL2, BCL2A1, BCL2L1, BCL2L10, BCL2L11, BCL2L13, BCL2L2, BID, BIK, BNIP3, BNIP3L, BOK, CASP3, CASP8, CASP9, CHCHD2, CHCHD3, CHCHD6, CYCS, DIABLO, DNAJC11, DNM1L, ENDOG, FIS1, FKBP8, FUNDC1, FUNDC2, GHITM, HTRA2, IFI27, IMMT, MARCHF5, MCL1, MFF, MFN1, MFN2, MGARP, MICOS10, MICOS13, MIEF1, MIEF2, MIGA1, MIGA2, MTCH2, MTFP1, MTFR1, MTFR2, MTX1, MTX2, MTX3, MUL1, MYO19, NBR1, NIPSNAP1, NIPSNAP2, OMA1, OPA1, PARK7, PARL, PGAM5, PIGBOS1, PINK1, PLD6, PMAIP1, PRKN, RAB24, RHOT1, RHOT2, RMDN3, RTL10, SAMM50, SEPTIN4, SLC25A46, SNAP29, SPHK2, SPIRE1, STX17, STYXL1, SYNJ2BP, TMEM11, TOMM20, TOMM7, USP30, VDAC1, VPS13D
FusionMitochondrial dynamics and surveillance > FusionARL2, MFN1, MFN2, MIGA1, MIGA2, MTCH2, OMA1, OPA1, PLD6
FissionMitochondrial dynamics and surveillance > FissionARMC10, DNM1L, FIS1, MFF, MIEF1, MIEF2, MTFP1, MTFR1, MTFR2, MUL1, OMA1, RAB24, SLC25A46, SPIRE1, STX17
Organelle contact sitesMitochondrial dynamics and surveillance > Organelle contact sitesAHCYL1, ATAD3A, ATAD3B, FKBP8, FUNDC2, MFN1, MFN2, MIGA2, PIGBOS1, RMDN3, SPIRE1, STX17, SYNJ2BP, VDAC1
Intramitochondrial membrane interactionsMitochondrial dynamics and surveillance > Intramitochondrial membrane interactionsATAD3A, ATAD3B, CHCHD3, DNAJC11, MTX1, MTX2, MTX3, SAMM50, SLC25A46
TraffickingMitochondrial dynamics and surveillance > TraffickingARMC10, ARMCX1, ARMCX3, MGARP, MYO19, RHOT1, RHOT2
MitophagyMitochondrial dynamics and surveillance > MitophagyBCL2L13, FKBP8, FUNDC1, MARCHF5, NIPSNAP1, NIPSNAP2, PARK7, PARL, PGAM5, PINK1, PRKN, TOMM7, USP30, VPS13D
AutophagyMitochondrial dynamics and surveillance > AutophagyBCL2L13, FKBP8, FUNDC1, MARCHF5, NBR1, NIPSNAP1, NIPSNAP2, PARK7, PARL, PGAM5, PINK1, PRKN, RAB24, SNAP29, STX17, TOMM7, USP30, VPS13D
ApoptosisMitochondrial dynamics and surveillance > ApoptosisAIFM1, AIFM2, AIFM3, BAD, BAK1, BAX, BBC3, BCL2, BCL2A1, BCL2L1, BCL2L10, BCL2L11, BCL2L13, BCL2L2, BID, BIK, BNIP3, BNIP3L, BOK, CASP3, CASP8, CASP9, CHCHD2, CYCS, DIABLO, ENDOG, GHITM, HTRA2, IFI27, MCL1, PMAIP1, RTL10, SEPTIN4, SPHK2, STYXL1
Cristae formationMitochondrial dynamics and surveillance > Cristae formationAPOO, APOOL, ATP5MD, ATP5ME, ATP5MG, CHCHD3, CHCHD6, IMMT, MICOS10, MICOS13, OPA1
MICOS complexMitochondrial dynamics and surveillance > Cristae formation > MICOS complexAPOO, APOOL, CHCHD3, CHCHD6, IMMT, MICOS10, MICOS13