Mitochondrial Biogenesis

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 Mitochondrial DNA transcription, translation, and replication factors
 
 Mitochondrial protein import machinery
 
 Mitochondrial quality control factors
   Regulator of mitochondrial biogenesis
     Ubiquitas transcription factors
     Nuclear respiratory factors
       K11831  NRF1; nuclear respiratory factor 1
       K05638  NFE2L2, NRF2; nuclear factor erythroid 2-related factor 2
       K14739  MT1_2; metallothionein 1/2
       K14740  MT3; metallothionein 3
       K14741  MT4; metallothionein 4
     Coactivators
       K07202  PPARGC1A, PGC1A; alpha peroxisome proliferator-activated receptor gamma coactivator 1-alpha
       K17962  PPARGC1B, PGC1B; peroxisome proliferator-activated receptor gamma coactivator 1-beta
       K17963  PPRC1, PRC; peroxisome proliferator-activated receptor gamma coactivator-related protein 1
       K15309  CRTC1, TORC1; CREB-regulated transcription coactivator 1
       K04498  EP300, CREBBP, KAT3; E1A/CREB-binding protein [EC:2.3.1.48]
     Proteins involved in Fe-S cluster biogenesis
     Other regulator of mitochondrial biogenesis
       K08765  CPT1A; carnitine O-palmitoyltransferase 1, liver isoform [EC:2.3.1.21]
       K19523  CPT1B; carnitine O-palmitoyltransferase 1, muscle isoform [EC:2.3.1.21]
       K19524  CPT1C; carnitine O-palmitoyltransferase 1, brain isoform [EC:2.3.1.21]
       K15791  DHKTD1; 2-oxoadipate dehydrogenase E1 component [EC:1.2.4.-]
       K10270  FBXL4; F-box and leucine-rich repeat protein 4
       K14966  HCFC1; host cell factor 1
       K27390  HCFC2; host cell factor 2
       K04533  HD; huntingtin
       K08552  NR3B1, ESRRA; estrogen-related receptor alpha
       K08553  NR3B2, ESRRB; estrogen-related receptor beta
       K08554  NR3B3, ESRRG; estrogen-related receptor gamma
       K17965  NRIP1; nuclear receptor-interacting protein 1
       K07198  PRKAA, AMPK; 5'-AMP-activated protein kinase, catalytic alpha subunit [EC:2.7.11.31]
       K08675  LONP1, PRSS15, PIM1; ATP-dependent Lon protease [EC:3.4.21.53]
       K11411  SIRT1, SIR2L1; NAD-dependent protein deacetylase sirtuin 1 [EC:2.3.1.286]
       K23504  SERAC1; protein SERAC1
       K26537  UXT; protein UXT
       K26738  PERM1; PGC-1 and ERR-induced regulator in muscle protein 1
       K27835  GATD3, ES1; glutamine amidotransferase-like class 1 domain-containing protein 3, mitochondrial
       K28098  TANGO2; transport and Golgi organization protein 2
       K28271  C2ORF69; mitochondrial protein C2orf69
       K28528  ATOS, FAM214; protein Atossa
       K14777  DDX47, RRP3; ATP-dependent RNA helicase DDX47/RRP3 [EC:5.6.2.7]
       K28918  PRR16; protein Largen
       K29035  MTLN; mitoregulin
       K18158  NCA2; nuclear control of ATPase protein 2
   Mitochondrial respiratory chain complex assembly factors
   Mitochondrial dynamics
     Fission and Fusion factors
       K21356  MFN1; mitofusin 1 [EC:3.6.5.-]
       K06030  MFN2, FZO1; mitofusin 2 [EC:3.6.5.-]
       K22140  MGM1; dynamin-like GTPase MGM1, mitochondrial
       K17967  UGO1; mitochondrial fusion and transport protein UGO1
       K17079  OPA1; optic atrophy protein 1 [EC:3.6.5.5]
       K23166  OPA3; optic atrophy 3 protein
       K15070  MDM30; mitochondrial distribution and morphology protein 30
       K17968  TRIAP1, MDM35; TRIAP1/MDM35 family protein
       K09650  PARL, PSARL, PCP1; rhomboid-like protein [EC:3.4.21.105]
       K14021  BAK, BAK1; Bcl-2 homologous antagonist/killer
       K02159  BAX; apoptosis regulator BAX
       K17065  DNM1L, DNM1; dynamin 1-like protein [EC:3.6.5.5]
       K17969  FIS1, TTC11, MDV2; mitochondrial fission 1 protein
       K17970  MDV1, FIS2; mitochondrial division protein 1
       K12160  SUMO, SMT3; small ubiquitin-related modifier
       K11248  SH3GLB1; endophilin-B1
       K17885  MTCH; mitochondrial carrier
       K22076  MFF; mitochondrial fission factor
       K22077  GDAP1; ganglioside-induced differentiation-associated protein 1
       K23167  VAT1; synaptic vesicle membrane protein VAT-1 [EC:1.6.5.5]
       K23507  MIEF1_2; mitochondrial dynamics protein MID51/49
       K16862  PLD6; mitochondrial cardiolipin hydrolase [EC:3.1.4.-]
       K27289  MIGA, FAM73; mitoguardin
       K27836  ELM1; mitochondrial fission protein ELM1
     Actin cytoskeleton organization
       K05692  ACTB_G1; actin beta/gamma 1
       K11238  BNI1; cytokinesis protein
       K17971  BNR1; BNI1-related protein 1
       K17945  TPM1_2; tropomyosin, fungi type
       K17972  NAA20, NAT3; N-terminal acetyltransferase B complex catalytic subunit [EC:2.3.1.254]
       K17973  NAA25, MDM20; N-terminal acetyltransferase B complex non-catalytic subunit
     Mitochondrial movement
     Retention of mitochondria
       K17976  MMR1; mitochondrial MYO2 receptor-related protein 1
       K17065  DNM1L, DNM1; dynamin 1-like protein [EC:3.6.5.5]
       K17977  YPT11; GTP-binding protein YPT11
       K10357  MYO5; myosin V
       K17978  NUM1; nuclear migration protein NUM1
       K04725  XIAP, BIRC4; E3 ubiquitin-protein ligase XIAP [EC:2.3.2.27]
       K10522  DIABLO, SMAC; diablo
       K28104  MME1; mitochondrial magnesium exporter 1
       K28103  MME1; mitochondrial magnesium exporter 1
     Morphogenesis factors
   Mitophagy factors
     Autophagy-related proteins
       K17906  ATG2; autophagy-related protein 2
       K08343  ATG3; ubiquitin-like-conjugating enzyme ATG3
       K08342  ATG4; cysteine protease ATG4 [EC:3.4.22.-]
       K08339  ATG5; autophagy-related protein 5
       K08334  BECN, VPS30, ATG6; beclin
       K08337  ATG7; ubiquitin-like modifier-activating enzyme ATG7
       K08341  GABARAP, ATG8, LC3; GABA(A) receptor-associated protein
       K17907  ATG9; autophagy-related protein 9
       K08338  ATG10; ubiquitin-like-conjugating enzyme ATG10
       K17888  ATG10L, ATG10; ubiquitin-like-conjugating enzyme ATG10
       K08330  ATG11; autophagy-related protein 11
       K08336  ATG12; ubiquitin-like protein ATG12
       K08335  ATG14; autophagy-related protein 14
       K17889  ATG14L, ATG14; beclin 1-associated autophagy-related key regulator
       K17900  ATG15, AUT5; lipase ATG15 [EC:3.1.1.3]
       K08340  ATG16; autophagy-related protein 16
       K17890  ATG16L1; autophagy-related protein 16-1
       K20868  ATG16L2; autophagy-related protein 16-2
       K08329  ATG17; autophagy-related protein 17
       K17908  WIPI1_2, ATG18; autophagy-related protein 18
       K17984  ATG32; autophagy-related protein 32
       K28529  ATG44; mitofissin
       K08332  VAC8; vacuolar protein 8
     Parkin-dependent mechanism factors
     Parkin-independent mechanism factors
       K08331  ATG13; autophagy-related protein 13
       K21357  ULK1; serine/threonine-protein kinase ULK1 [EC:2.7.11.1]
       K08269  ULK2, ATG1; serine/threonine-protein kinase ULK2 [EC:2.7.11.1]
       K17589  RB1CC1; RB1-inducible coiled-coil protein 1
       K04079  HSP90A, htpG; molecular chaperone HtpG
       K09554  CDC37; cell division cycle protein 37
       K04678  SMURF; E3 ubiquitin ligase SMURF1/2 [EC:2.3.2.26]
       K15465  BNIP3L; BCL2/adenovirus E1B 19 kDa protein-interacting protein 3-like
       K17986  FUNDC1; FUN14 domain-containing protein 1
     Other mitophagy factors

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Last updated: July 24, 2026