 Mitochondrial DNA transcription, translation, and replication factors
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 Mitochondrial DNA-encoded proteins
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Mitochondrial transcription and translation factors
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Mitochondrial RNA polymerase
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Mitochondrial transcription factors
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Mitochondrial translation factors
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RNase MRP
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Mitochondrial ribonuclease P
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Other mitochondrial DNA transcription and translation factors
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 K17656 MSS51; mitochondrial splicing suppressor protein 51
K17657 MRS1; mitochondrial RNA-splicing protein MRS1
K17658 TEFM; transcription elongation factor, mitochondrial
K17710 PTCD1; pentatricopeptide repeat domain-containing protein 1
K17711 PTCD2; pentatricopeptide repeat domain-containing protein 2
K17659 PTCD3, MRPS39; pentatricopeptide repeat domain-containing protein 3 (small subunit ribosomal protein S39)
K13185 DHX30; ATP-dependent RNA helicase DHX30 [EC:5.6.2.6]
K17660 CBP1; cytochrome b pre-mRNA-processing protein 1
K17661 CBP2; cytochrome b pre-mRNA-processing protein 2
K17662 CBP3, UQCC; cytochrome b pre-mRNA-processing protein 3
K17663 CBP6; cytochrome b pre-mRNA-processing protein 6
K17664 CBS1; cytochrome b translational activator protein CBS1, mitochondrial
K17665 CBS2; cytochrome b translational activator protein CBS2, mitochondrial
K17666 PET54; protein PET54, mitochondrial
K17667 PET111; protein PET111, mitochondrial
K17668 PET122; protein PET122, mitochondrial
K17669 PET309; pentatricopeptide repeat-containing protein PET309
K17670 PET494; COX3 mRNA-specific translational activator PET494
K17671 AEP1; ATPase expression protein 1, mitochondrial
K17672 AEP2; ATPase expression protein 2, mitochondrial
K18155 AEP3; ATPase expression protein 3, mitochondrial
K18798 AFG1, LACE1; peroxisome-assembly ATPase [EC:3.6.4.7]
K17673 ATP22; mitochondrial translation factor ATP22
K18156 ATP23, XRCC6BP1; mitochondrial inner membrane protease ATP23 [EC:3.4.24.-]
K17674 SOV1; protein SOV1, mitochondrial
K17675 SUPV3L1, SUV3; ATP-dependent RNA helicase SUPV3L1/SUV3 [EC:5.6.2.6]
K19782 DSS1, MSU1; exoribonuclease II, mitochondrial [EC:3.1.13.1]
K16639 LACTB2; endoribonuclease LACTB2 [EC:3.1.27.-]
K05863 SLC25A4S, ANT; solute carrier family 25 (mitochondrial adenine nucleotide translocator), member 4/5/6/31
K15113 SLC25A28_37, MFRN; solute carrier family 25 (mitochondrial iron transporter), member 28/37
K25803 MALSU1; mitochondrial assembly of ribosomal large subunit protein 1
K03595 era, ERAL1; GTPase
K01866 YARS, tyrS; tyrosyl-tRNA synthetase [EC:6.1.1.1]
K01869 LARS, leuS; leucyl-tRNA synthetase [EC:6.1.1.4]
K01876 DARS2, aspS; aspartyl-tRNA synthetase [EC:6.1.1.12]
K01880 GARS, glyS1; glycyl-tRNA synthetase [EC:6.1.1.14]
K01887 RARS, argS; arginyl-tRNA synthetase [EC:6.1.1.19]
K02433 gatA, QRSL1; aspartyl-tRNA(Asn)/glutamyl-tRNA(Gln) amidotransferase subunit A [EC:6.3.5.6 6.3.5.7]
K02434 gatB, PET112; aspartyl-tRNA(Asn)/glutamyl-tRNA(Gln) amidotransferase subunit B [EC:6.3.5.6 6.3.5.7]
K02435 gatC, GATC; aspartyl-tRNA(Asn)/glutamyl-tRNA(Gln) amidotransferase subunit C [EC:6.3.5.6 6.3.5.7]
K18060 MTPAP; poly(A) RNA polymerase, mitochondrial [EC:2.7.7.19]
K19612 PDE12; 2',5'-phosphodiesterase [EC:3.1.13.4 3.1.4.-]
K19828 MTG1; mitochondrial GTPase 1
K19832 NOA1; nitric oxide-associated protein 1
K13427 NOA1; nitric-oxide synthase, plant [EC:1.14.13.39]
K21969 NSUN3; tRNA (cytosine34-C5)-methyltransferase [EC:2.1.1.-]
K21970 NSUN4; 5-methylcytosine rRNA methyltransferase NSUN4 [EC:2.1.1.-]
K15452 PUS2; mitochondrial tRNA pseudouridine27/28 synthase [EC:5.4.99.44]
K22537 RPUSD3; mitochondrial RNA pseudouridine synthase RPUSD3 [EC:5.4.99.-]
K22538 RPUSD4; mitochondrial RNA pseudouridine synthase RPUSD4 [EC:5.4.99.-]
K23495 RCC1L; RCC1-like G exchanging factor-like protein
K23496 NGRN; neugrin
K23497 TRUB2; mitochondrial mRNA pseudouridine synthase TRUB2 [EC:5.4.99.-]
K27754 FASTKD1; FAST kinase domain-containing protein 1
K18190 FASTKD2; FAST kinase domain-containing protein 2
K27755 FASTKD3; FAST kinase domain-containing protein 3
K27756 TBRG4, FASTKD4; FAST kinase domain-containing protein 4
K27757 FASTKD5; FAST kinase domain-containing protein 5
K23498 COXPD7; peptide chain release factor
K23499 RMND1; required for meiotic nuclear division protein 1
K24984 GRSF1; G-rich sequence factor 1
K25876 METTL15; 12S rRNA N4-methylcytidine (m4C) methyltransferase [EC:2.1.1.-]
K25877 MYG1; MYG1 exonuclease [EC:3.1.-.-]
K26186 MTRES1; mitochondrial transcription rescue factor 1
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Mitochondrial DNA replication factors
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Mitochondrial protein import machinery
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Mitochondrial quality control factors
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Regulator of mitochondrial biogenesis
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Ubiquitas transcription factors
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Nuclear respiratory factors
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Coactivators
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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]
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Proteins involved in Fe-S cluster biogenesis
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Other regulator of mitochondrial biogenesis
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Mitochondrial respiratory chain complex assembly factors
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Complex-I assembly factors
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Complex-II assembly factors
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Complex-III assembly factors
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K08900 BCS1; mitochondrial chaperone BCS1
K18169 TTC19; tetratricopeptide repeat protein 19, mitochondrial
K18170 LYRM7, MZM1; complex III assembly factor LYRM7
K17662 CBP3, UQCC; cytochrome b pre-mRNA-processing protein 3
K17682 MNF1; mitochondrial nucleoid factor 1
K23165 UQCC3; ubiquinol-cytochrome-c reductase complex assembly factor 3
K27339 UQCC4; ubiquinol-cytochrome c reductase complex assembly factor 4
K27340 UQCC5, SMIM4; ubiquinol-cytochrome c reductase complex assembly factor 5
K27341 UQCC6; ubiquinol-cytochrome c reductase complex assembly factor 6
K27319 STMP1; short transmembrane mitochondrial protein 1
K29036 OCIAD2; OCIA domain-containing protein 2
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Complex-IV assembly factors
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Complex-V assembly factors
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Mitochondrial dynamics
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Fission and Fusion factors
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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
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
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Actin cytoskeleton organization
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Mitochondrial movement
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Retention of mitochondria
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Morphogenesis factors
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Mitophagy factors
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Mitochondrial Biogenesis