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Mitochondrial Biogenesis - Homo sapiens (human)
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AMitochondrial DNA transcription, translation, and replication factors
B Mitochondrial DNA-encoded proteins
C Mitochondrial respiratory chain complex I
D 4535 ND1; NADH dehydrogenase subunit 1 K03878 ND1; NADH-ubiquinone oxidoreductase chain 1 [EC:7.1.1.2]
D 4536 ND2; NADH dehydrogenase subunit 2 K03879 ND2; NADH-ubiquinone oxidoreductase chain 2 [EC:7.1.1.2]
D 4537 ND3; NADH dehydrogenase subunit 3 K03880 ND3; NADH-ubiquinone oxidoreductase chain 3 [EC:7.1.1.2]
D 4538 ND4; NADH dehydrogenase subunit 4 K03881 ND4; NADH-ubiquinone oxidoreductase chain 4 [EC:7.1.1.2]
D 4539 ND4L; NADH dehydrogenase subunit 4L K03882 ND4L; NADH-ubiquinone oxidoreductase chain 4L [EC:7.1.1.2]
D 4540 ND5; NADH dehydrogenase subunit 5 K03883 ND5; NADH-ubiquinone oxidoreductase chain 5 [EC:7.1.1.2]
D 4541 ND6; NADH dehydrogenase subunit 6 K03884 ND6; NADH-ubiquinone oxidoreductase chain 6 [EC:7.1.1.2]
C Mitochondrial respiratory chain complex III
D 4519 CYTB; cytochrome b K00412 CYTB; ubiquinol-cytochrome c reductase cytochrome b subunit
C Cytochrome c oxidase
D 4512 COX1; cytochrome c oxidase subunit I K02256 COX1; cytochrome c oxidase subunit 1 [EC:7.1.1.9]
D 4513 COX2; cytochrome c oxidase subunit II K02261 COX2; cytochrome c oxidase subunit 2
D 4514 COX3; cytochrome c oxidase subunit III K02262 COX3; cytochrome c oxidase subunit 3
C F-type ATPase
D 4509 ATP8; ATP synthase F0 subunit 8 K02125 ATPeF08; F-type H+-transporting ATPase subunit 8
D 4508 ATP6; ATP synthase F0 subunit 6 K02126 ATPeF0A; F-type H+-transporting ATPase subunit a
B Mitochondrial transcription and translation factors
C Mitochondrial RNA polymerase
D 5442 POLRMT; RNA polymerase mitochondrial K10908 POLRMT; DNA-directed RNA polymerase, mitochondrial [EC:2.7.7.6]
C Mitochondrial transcription factors
D 7019 TFAM; transcription factor A, mitochondrial K11830 TFAM; transcription factor A, mitochondrial
D 51106 TFB1M; transcription factor B1, mitochondrial K15266 TFB1M; dimethyladenosine transferase 1, mitochondrial [EC:2.1.1.-]
D 64216 TFB2M; transcription factor B2, mitochondrial K17653 TFB2M; dimethyladenosine transferase 2, mitochondrial [EC:2.1.1.-]
C Mitochondrial translation factors
D 4528 MTIF2; mitochondrial translational initiation factor 2 K02519 infB; translation initiation factor IF-2
D 219402 MTIF3; mitochondrial translational initiation factor 3 K02520 infC; translation initiation factor IF-3
D 10102 TSFM; Ts translation elongation factor, mitochondrial K02357 tsf; elongation factor Ts
D 7284 TUFM; Tu translation elongation factor, mitochondrial K02358 tuf; elongation factor Tu
D 84340 GFM2; GTP dependent ribosome recycling factor mitochondrial 2 K02355 fusA; elongation factor G
D 85476 GFM1; G elongation factor mitochondrial 1 K02355 fusA; elongation factor G
D 7978 MTERF1; mitochondrial transcription termination factor 1 K15031 MTERF; mitochondrial transcription termination factor family protein
D 80298 MTERF2; mitochondrial transcription termination factor 2 K15031 MTERF; mitochondrial transcription termination factor family protein
D 130916 MTERF4; mitochondrial transcription termination factor 4 K15031 MTERF; mitochondrial transcription termination factor family protein
D 51001 MTERF3; mitochondrial transcription termination factor 3 K15031 MTERF; mitochondrial transcription termination factor family protein
D 60558 GUF1; GTP binding elongation factor GUF1 K21594 GUF1; translation factor GUF1, mitochondrial [EC:3.6.5.-]
C RNase MRP
D 10940 POP1; POP1 homolog, ribonuclease P/MRP subunit K01164 POP1; ribonuclease P/MRP protein subunit POP1 [EC:3.1.26.5]
D 10775 POP4; POP4 homolog, ribonuclease P/MRP subunit K03538 POP4; ribonuclease P protein subunit POP4 [EC:3.1.26.5]
D 51367 POP5; POP5 homolog, ribonuclease P/MRP subunit K03537 POP5; ribonuclease P/MRP protein subunit POP5 [EC:3.1.26.5]
D 10556 RPP30; ribonuclease P/MRP subunit p30 K03539 RPP1; ribonuclease P/MRP protein subunit RPP1 [EC:3.1.26.5]
D 10248 POP7; POP7 homolog, ribonuclease P/MRP subunit K14527 RPP20; ribonuclease P/MRP protein subunit RPP20 [EC:3.1.26.5]
D 138716 RPP25L; ribonuclease P/MRP subunit p25 like K14525 RPP25; ribonucleases P/MRP protein subunit RPP25 [EC:3.1.26.5]
D 54913 RPP25; ribonuclease P and MRP subunit p25 K14525 RPP25; ribonucleases P/MRP protein subunit RPP25 [EC:3.1.26.5]
D 10557 RPP38; ribonuclease P/MRP subunit p38 K14523 RPP38; ribonucleases P/MRP protein subunit RPP38 [EC:3.1.26.5]
D 10799 RPP40; ribonuclease P/MRP subunit p40 K14530 RPP40; ribonucleases P/MRP protein subunit RPP40 [EC:3.1.26.5]
C Mitochondrial ribonuclease P
D 54931 TRMT10C; tRNA methyltransferase 10C, mitochondrial RNase P subunit K17654 TRMT10C; mitochondrial ribonuclease P protein 1 [EC:2.1.1.-]
D 3028 HSD17B10; hydroxysteroid 17-beta dehydrogenase 10 K08683 HSD17B10; 3-hydroxyacyl-CoA dehydrogenase / 3-hydroxy-2-methylbutyryl-CoA dehydrogenase [EC:1.1.1.35 1.1.1.178]
D 9692 PRORP; protein only RNase P catalytic subunit K17655 MRPP3; mitochondrial ribonuclease P protein 3
C Other mitochondrial DNA transcription and translation factors
D 118490 MSS51; MSS51 mitochondrial translational activator K17656 MSS51; mitochondrial splicing suppressor protein 51
D 79736 TEFM; transcription elongation factor, mitochondrial K17658 TEFM; transcription elongation factor, mitochondrial
D 100526740 ATP5MF-PTCD1; ATP5MF-PTCD1 readthrough K17710 PTCD1; pentatricopeptide repeat domain-containing protein 1
D 26024 PTCD1; pentatricopeptide repeat domain 1 K17710 PTCD1; pentatricopeptide repeat domain-containing protein 1
D 79810 PTCD2; pentatricopeptide repeat domain 2 K17711 PTCD2; pentatricopeptide repeat domain-containing protein 2
D 55037 PTCD3; pentatricopeptide repeat domain 3 K17659 PTCD3; pentatricopeptide repeat domain-containing protein 3
D 22907 DHX30; DExH-box helicase 30 K13185 DHX30; ATP-dependent RNA helicase DHX30 [EC:5.6.2.6]
D 55245 UQCC1; ubiquinol-cytochrome c reductase complex assembly factor 1 K17662 CBP3; cytochrome b pre-mRNA-processing protein 3
D 246269 AFG1L; AFG1 like ATPase K18798 AFG1; peroxisome-assembly ATPase [EC:3.6.4.7]
D 91419 ATP23; ATP23 metallopeptidase and ATP synthase assembly factor homolog K18156 ATP23; mitochondrial inner membrane protease ATP23 [EC:3.4.24.-]
D 6832 SUPV3L1; Suv3 like RNA helicase K17675 SUPV3L1; ATP-dependent RNA helicase SUPV3L1/SUV3 [EC:5.6.2.6]
D 51110 LACTB2; lactamase beta 2 K16639 LACTB2; endoribonuclease LACTB2 [EC:3.1.27.-]
D 291 SLC25A4; solute carrier family 25 member 4 K05863 SLC25A4S; solute carrier family 25 (mitochondrial adenine nucleotide translocator), member 4/5/6/31
D 292 SLC25A5; solute carrier family 25 member 5 K05863 SLC25A4S; solute carrier family 25 (mitochondrial adenine nucleotide translocator), member 4/5/6/31
D 293 SLC25A6; solute carrier family 25 member 6 K05863 SLC25A4S; solute carrier family 25 (mitochondrial adenine nucleotide translocator), member 4/5/6/31
D 83447 SLC25A31; solute carrier family 25 member 31 K05863 SLC25A4S; solute carrier family 25 (mitochondrial adenine nucleotide translocator), member 4/5/6/31
D 51312 SLC25A37; solute carrier family 25 member 37 K15113 SLC25A28_37; solute carrier family 25 (mitochondrial iron transporter), member 28/37
D 81894 SLC25A28; solute carrier family 25 member 28 K15113 SLC25A28_37; solute carrier family 25 (mitochondrial iron transporter), member 28/37
D 115416 MALSU1; mitochondrial assembly of ribosomal large subunit 1 K25803 MALSU1; mitochondrial assembly of ribosomal large subunit protein 1
D 26284 ERAL1; Era like 12S mitochondrial rRNA chaperone 1 K03595 era; GTPase
D 8565 YARS1; tyrosyl-tRNA synthetase 1 K01866 YARS; tyrosyl-tRNA synthetase [EC:6.1.1.1]
D 51067 YARS2; tyrosyl-tRNA synthetase 2 K01866 YARS; tyrosyl-tRNA synthetase [EC:6.1.1.1]
D 51520 LARS1; leucyl-tRNA synthetase 1 K01869 LARS; leucyl-tRNA synthetase [EC:6.1.1.4]
D 23395 LARS2; leucyl-tRNA synthetase 2, mitochondrial K01869 LARS; leucyl-tRNA synthetase [EC:6.1.1.4]
D 55157 DARS2; aspartyl-tRNA synthetase 2, mitochondrial K01876 DARS2; aspartyl-tRNA synthetase [EC:6.1.1.12]
D 2617 GARS1; glycyl-tRNA synthetase 1 K01880 GARS; glycyl-tRNA synthetase [EC:6.1.1.14]
D 57038 RARS2; arginyl-tRNA synthetase 2, mitochondrial K01887 RARS; arginyl-tRNA synthetase [EC:6.1.1.19]
D 5917 RARS1; arginyl-tRNA synthetase 1 K01887 RARS; arginyl-tRNA synthetase [EC:6.1.1.19]
D 55278 QRSL1; glutaminyl-tRNA amidotransferase subunit QRSL1 K02433 gatA; aspartyl-tRNA(Asn)/glutamyl-tRNA(Gln) amidotransferase subunit A [EC:6.3.5.6 6.3.5.7]
D 5188 GATB; glutamyl-tRNA amidotransferase subunit B K02434 gatB; aspartyl-tRNA(Asn)/glutamyl-tRNA(Gln) amidotransferase subunit B [EC:6.3.5.6 6.3.5.7]
D 283459 GATC; glutamyl-tRNA amidotransferase subunit C K02435 gatC; aspartyl-tRNA(Asn)/glutamyl-tRNA(Gln) amidotransferase subunit C [EC:6.3.5.6 6.3.5.7]
D 55149 MTPAP; mitochondrial poly(A) polymerase K18060 MTPAP; poly(A) RNA polymerase, mitochondrial [EC:2.7.7.19]
D 201626 PDE12; phosphodiesterase 12 K19612 PDE12; 2',5'-phosphodiesterase [EC:3.1.13.4 3.1.4.-]
D 92170 MTG1; mitochondrial ribosome associated GTPase 1 K19828 MTG1; mitochondrial GTPase 1
D 84273 NOA1; nitric oxide associated 1 K19832 NOA1; nitric oxide-associated protein 1
D 63899 NSUN3; NOP2/Sun RNA methyltransferase 3 K21969 NSUN3; tRNA (cytosine34-C5)-methyltransferase [EC:2.1.1.-]
D 387338 NSUN4; NOP2/Sun RNA methyltransferase 4 K21970 NSUN4; 5-methylcytosine rRNA methyltransferase NSUN4 [EC:2.1.1.-]
D 285367 RPUSD3; RNA pseudouridine synthase D3 K22537 RPUSD3; mitochondrial RNA pseudouridine synthase RPUSD3 [EC:5.4.99.-]
D 84881 RPUSD4; RNA pseudouridine synthase D4 K22538 RPUSD4; mitochondrial RNA pseudouridine synthase RPUSD4 [EC:5.4.99.-]
D 81554 RCC1L; RCC1 like K23495 RCC1L; RCC1-like G exchanging factor-like protein
D 51335 NGRN; neugrin, neurite outgrowth associated K23496 NGRN; neugrin
D 26995 TRUB2; TruB pseudouridine synthase family member 2 K23497 TRUB2; mitochondrial mRNA pseudouridine synthase TRUB2 [EC:5.4.99.-]
D 79675 FASTKD1; FAST kinase domains 1 K27754 FASTKD1; FAST kinase domain-containing protein 1
D 22868 FASTKD2; FAST kinase domains 2 K18190 FASTKD2; FAST kinase domain-containing protein 2
D 79072 FASTKD3; FAST kinase domains 3 K27755 FASTKD3; FAST kinase domain-containing protein 3
D 9238 TBRG4; transforming growth factor beta regulator 4 K27756 TBRG4; FAST kinase domain-containing protein 4
D 60493 FASTKD5; FAST kinase domains 5 K27757 FASTKD5; FAST kinase domain-containing protein 5
D 91574 MTRFR; mitochondrial translation release factor in rescue K23498 COXPD7; peptide chain release factor
D 55005 RMND1; required for meiotic nuclear division 1 homolog K23499 RMND1; required for meiotic nuclear division protein 1
D 2926 GRSF1; G-rich RNA sequence binding factor 1 K24984 GRSF1; G-rich sequence factor 1
D 196074 METTL15; methyltransferase 15, mitochondrial 12S rRNA N4-cytidine K25876 METTL15; 12S rRNA N4-methylcytidine (m4C) methyltransferase [EC:2.1.1.-]
D 60314 MYG1; MYG1 exonuclease K25877 MYG1; MYG1 exonuclease [EC:3.1.-.-]
D 51250 MTRES1; mitochondrial transcription rescue factor 1 K26186 MTRES1; mitochondrial transcription rescue factor 1
B Mitochondrial DNA replication factors
C Mitochondrial DNA polymerase
D 5428 POLG; DNA polymerase gamma, catalytic subunit K02332 POLG; DNA polymerase gamma 1 [EC:2.7.7.7]
D 11232 POLG2; DNA polymerase gamma 2, accessory subunit K02333 POLG2; DNA polymerase gamma 2
D 201973 PRIMPOL; primase and DNA directed polymerase K22761 PRIMPOL; DNA-directed primase/polymerase protein [EC:2.7.7.102 2.7.7.7]
C Mitochondrial DNA maintenance factors
D 2021 ENDOG; endonuclease G K01173 ENDOG; endonuclease G, mitochondrial
D 92667 MGME1; mitochondrial genome maintenance exonuclease 1 K19465 MGME1; mitochondrial genome maintenance exonuclease 1 [EC:3.1.-.-]
D 9941 EXOG; exo/endonuclease G K15050 EXOG; nuclease EXOG, mitochondrial
D 56652 TWNK; twinkle mtDNA helicase K17680 PEO1; twinkle protein [EC:5.6.2.3]
D 80119 PIF1; PIF1 5'-to-3' DNA helicase K15255 PIF1; ATP-dependent DNA helicase PIF1 [EC:5.6.2.3]
C Other Mitochondrial DNA replication factors
D 6742 SSBP1; single stranded DNA binding protein 1 K03111 ssb; single-strand DNA-binding protein
D 83858 ATAD3B; ATPase family AAA domain containing 3B K17681 ATAD3A_B; ATPase family AAA domain-containing protein 3A/B
D 55210 ATAD3A; ATPase family AAA domain containing 3A K17681 ATAD3A_B; ATPase family AAA domain-containing protein 3A/B
D 84300 UQCC2; ubiquinol-cytochrome c reductase complex assembly factor 2 K17682 MNF1; mitochondrial nucleoid factor 1
D 55028 MTNAP1; mitochondrial nucleoid associated protein 1 K27759 MTNAP1; mitochondrial nucleoid-associated protein 1
D 55340 GIMAP5; GTPase, IMAP family member 5 K27758 GIMAP1_3_5; GTPase IMAP family member 1/3/5
D 170575 GIMAP1; GTPase, IMAP family member 1 K27758 GIMAP1_3_5; GTPase IMAP family member 1/3/5
D 100527949 GIMAP1-GIMAP5; GIMAP1-GIMAP5 readthrough K27758 GIMAP1_3_5; GTPase IMAP family member 1/3/5
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AMitochondrial protein import machinery
B Endoplasmic reticulum membrane and cytosol
C ER-mitochondria encounter structure (ERMES) complex
C Other ER-memrane and cytosol factors
D 10294 DNAJA2; DnaJ heat shock protein family (Hsp40) member A2 K09503 DNAJA2; DnaJ homolog subfamily A member 2
D 10953 TOMM34; translocase of outer mitochondrial membrane 34 K17766 TOM34; mitochondrial import receptor subunit TOM34
D 55288 RHOT1; ras homolog family member T1 K07870 RHOT1; mitochondrial Rho GTPase 1 [EC:3.6.5.-]
D 3312 HSPA8; heat shock protein family A (Hsp70) member 8 K03283 HSPA1_6_8; heat shock 70kDa protein 1/6/8
D 3303 HSPA1A; heat shock protein family A (Hsp70) member 1A K03283 HSPA1_6_8; heat shock 70kDa protein 1/6/8
D 3305 HSPA1L; heat shock protein family A (Hsp70) member 1 like K03283 HSPA1_6_8; heat shock 70kDa protein 1/6/8
D 3304 HSPA1B; heat shock protein family A (Hsp70) member 1B K03283 HSPA1_6_8; heat shock 70kDa protein 1/6/8
D 3310 HSPA6; heat shock protein family A (Hsp70) member 6 K03283 HSPA1_6_8; heat shock 70kDa protein 1/6/8
D 23746 AIPL1; aryl hydrocarbon receptor interacting protein like 1 K17767 AIP; AH receptor-interacting protein
D 9049 AIP; aryl hydrocarbon receptor interacting protein K17767 AIP; AH receptor-interacting protein
B Outer membrane
C Transporter outer membrane (TOM) complex
D 10452 TOMM40; translocase of outer mitochondrial membrane 40 K11518 TOM40; mitochondrial import receptor subunit TOM40
D 84134 TOMM40L; translocase of outer mitochondrial membrane 40 like K11518 TOM40; mitochondrial import receptor subunit TOM40
D 9868 TOMM70; translocase of outer mitochondrial membrane 70 K17768 TOM70; mitochondrial import receptor subunit TOM70
D 56993 TOMM22; translocase of outer mitochondrial membrane 22 K17769 TOM22; mitochondrial import receptor subunit TOM22
D 387990 TOMM20L; translocase of outer mitochondrial membrane 20 like K17770 TOM20; mitochondrial import receptor subunit TOM20
D 9804 TOMM20; translocase of outer mitochondrial membrane 20 K17770 TOM20; mitochondrial import receptor subunit TOM20
D 54543 TOMM7; translocase of outer mitochondrial membrane 7 K17771 TOM7; mitochondrial import receptor subunit TOM7
D 107986346 mitochondrial import receptor subunit TOM7 homolog K17771 TOM7; mitochondrial import receptor subunit TOM7
D 100188893 TOMM6; translocase of outer mitochondrial membrane 6 K17772 TOM6; mitochondrial import receptor subunit TOM6
D 401505 TOMM5; translocase of outer mitochondrial membrane 5 K17773 TOM5; mitochondrial import receptor subunit TOM5
D 128966712 mitochondrial import receptor subunit TOM5 homolog K17773 TOM5; mitochondrial import receptor subunit TOM5
D 128966726 mitochondrial import receptor subunit TOM5 homolog K17773 TOM5; mitochondrial import receptor subunit TOM5
D 107986372 mitochondrial import receptor subunit TOM5 homolog K17773 TOM5; mitochondrial import receptor subunit TOM5
D 107986373 mitochondrial import receptor subunit TOM5 homolog K17773 TOM5; mitochondrial import receptor subunit TOM5
C TOB/SAM complex
D 25813 SAMM50; SAMM50 sorting and assembly machinery component K07277 SAM50; outer membrane protein insertion porin family
C Porin
D 7416 VDAC1; voltage dependent anion channel 1 K05862 VDAC1; voltage-dependent anion channel protein 1
D 7417 VDAC2; voltage dependent anion channel 2 K15040 VDAC2; voltage-dependent anion channel protein 2
D 7419 VDAC3; voltage dependent anion channel 3 K15041 VDAC3; voltage-dependent anion channel protein 3
C ERMES complex
C Other outer membrane factor
D 345778 MTX3; metaxin 3 K17776 MTX; metaxin
D 4580 MTX1; metaxin 1 K17776 MTX; metaxin
D 10651 MTX2; metaxin 2 K17776 MTX; metaxin
D 80179 MYO19; myosin XIX K25838 MYO19; myosin XIX
D 51309 ARMCX1; armadillo repeat containing X-linked 1 K26187 ARMCX1; armadillo repeat-containing X-linked protein 1
D 9823 ARMCX2; armadillo repeat containing X-linked 2 K26188 ARMCX2; armadillo repeat-containing X-linked protein 2
D 51566 ARMCX3; armadillo repeat containing X-linked 3 K26189 ARMCX3; armadillo repeat-containing X-linked protein 3
D 54470 ARMCX6; armadillo repeat containing X-linked 6 K26190 ARMCX6; armadillo repeat-containing X-linked protein 6
D 64757 MTARC1; mitochondrial amidoxime reducing component 1 K27318 MTARC; mitochondrial amidoxime-reducing component [EC:1.7.-.-]
D 54996 MTARC2; mitochondrial amidoxime reducing component 2 K27318 MTARC; mitochondrial amidoxime-reducing component [EC:1.7.-.-]
B Inter membrane space
C Transporter inner membrane (TIM) 9/10 complex
D 26520 TIMM9; translocase of inner mitochondrial membrane 9 K17777 TIM9; mitochondrial import inner membrane translocase subunit TIM9
D 26519 TIMM10; translocase of inner mitochondrial membrane 10 K17778 TIM10; mitochondrial import inner membrane translocase subunit TIM10
D 26515 TIMM10B; translocase of inner mitochondrial membrane 10B K17779 TIM10B; mitochondrial import inner membrane translocase subunit TIM10B
C TIM8/13 complex
D 1678 TIMM8A; translocase of inner mitochondrial membrane 8A K17780 TIM8; mitochondrial import inner membrane translocase subunit TIM8
D 26521 TIMM8B; translocase of inner mitochondrial membrane 8 homolog B K17780 TIM8; mitochondrial import inner membrane translocase subunit TIM8
D 26517 TIMM13; translocase of inner mitochondrial membrane 13 K17781 TIM13; mitochondrial import inner membrane translocase subunit TIM13
C Mitochondrial intermembrane space assembly (MIA) complex
D 131474 CHCHD4; coiled-coil-helix-coiled-coil-helix domain containing 4 K17782 MIA40; mitochondrial intermembrane space import and assembly protein 40
D 2671 GFER; growth factor, augmenter of liver regeneration K17783 ERV1; mitochondrial FAD-linked sulfhydryl oxidase [EC:1.8.3.2]
B Inner mambrane
C MICOS complex
D 440574 MICOS10; mitochondrial contact site and cristae organizing system subunit 10 K17784 MICOS10; MICOS complex subunit MIC10
D 10989 IMMT; inner membrane mitochondrial protein K17785 IMMT; MICOS complex subunit MIC60
D 125988 MICOS13; mitochondrial contact site and cristae organizing system subunit 13 K24624 MICOS13; MICOS complex subunit MIC13
D 54927 CHCHD3; coiled-coil-helix-coiled-coil-helix domain containing 3 K17563 CHCHD3; MICOS complex subunit MIC19
D 84303 CHCHD6; coiled-coil-helix-coiled-coil-helix domain containing 6 K17564 CHCHD6; MICOS complex subunit MIC25
D 79135 APOO; apolipoprotein O K24625 APOO; MICOS complex subunit MIC26
D 139322 APOOL; apolipoprotein O like K24626 APOOL; MICOS complex subunit MIC27
C MICOS complex (yeast)
D 440574 MICOS10; mitochondrial contact site and cristae organizing system subunit 10 K17784 MICOS10; MICOS complex subunit MIC10
D 10989 IMMT; inner membrane mitochondrial protein K17785 IMMT; MICOS complex subunit MIC60
C TIM22 complex
D 29928 TIMM22; translocase of inner mitochondrial membrane 22 K17790 TIM22; mitochondrial import inner membrane translocase subunit TIM22
C TIM23 complex
D 100287932 TIMM23; translocase of inner mitochondrial membrane 23 K17794 TIM23; mitochondrial import inner membrane translocase subunit TIM23
D 100652748 TIMM23B; translocase of inner mitochondrial membrane 23 homolog B K17794 TIM23; mitochondrial import inner membrane translocase subunit TIM23
D 10245 TIMM17B; translocase of inner mitochondrial membrane 17B K17795 TIM17; mitochondrial import inner membrane translocase subunit TIM17
D 10440 TIMM17A; translocase of inner mitochondrial membrane 17A K17795 TIM17; mitochondrial import inner membrane translocase subunit TIM17
D 29090 TIMM21; translocase of inner mitochondrial membrane 21 K17796 TIM21; mitochondrial import inner membrane translocase subunit TIM21
D 92609 TIMM50; translocase of inner mitochondrial membrane 50 K17496 TIM50; mitochondrial import inner membrane translocase subunit TIM50
C Mitochondrial calcium uniporter complex
D 90550 MCU; mitochondrial calcium uniporter K20858 MCU; calcium uniporter protein, mitochondrial
D 10367 MICU1; mitochondrial calcium uptake 1 K22827 MICU1; calcium uptake protein 1, mitochondrial
D 221154 MICU2; mitochondrial calcium uptake 2 K22828 MICU2; calcium uptake protein 2, mitochondrial
D 55013 MCUB; mitochondrial calcium uniporter dominant negative subunit beta K22829 MCUB; calcium uniporter regulatory subunit MCUb, mitochondrial
D 91689 SMDT1; single-pass membrane protein with aspartate rich tail 1 K22830 SMDT1; essential MCU regulator, mitochondrial
D 63933 MCUR1; mitochondrial calcium uniporter regulator 1 K22137 MCUR1; mitochondrial calcium uniporter regulator 1
C Other inner membrane factors
D 3954 LETM1; leucine zipper and EF-hand containing transmembrane protein 1 K17800 LETM1; LETM1 and EF-hand domain-containing protein 1, mitochondrial
D 196294 IMMP1L; inner mitochondrial membrane peptidase subunit 1 K09647 IMP1; mitochondrial inner membrane protease subunit 1 [EC:3.4.21.-]
D 83943 IMMP2L; inner mitochondrial membrane peptidase subunit 2 K09648 IMP2; mitochondrial inner membrane protease subunit 2 [EC:3.4.21.-]
D 84233 TMEM126A; transmembrane protein 126A K18157 TMEM126A; transmembrane protein 126A
D 347411 MPC1L; mitochondrial pyruvate carrier 1 like K22138 MPC1; mitochondrial pyruvate carrier 1
D 51660 MPC1; mitochondrial pyruvate carrier 1 K22138 MPC1; mitochondrial pyruvate carrier 1
D 25874 MPC2; mitochondrial pyruvate carrier 2 K22139 MPC2; mitochondrial pyruvate carrier 2
D 400916 CHCHD10; coiled-coil-helix-coiled-coil-helix domain containing 10 K22759 CHCHD10; coiled-coil-helix-coiled-coil-helix domain-containing protein 10
B Matrix
C MMC/PAM proteins
D 10469 TIMM44; translocase of inner mitochondrial membrane 44 K17804 TIM44; mitochondrial import inner membrane translocase subunit TIM44
D 3313 HSPA9; heat shock protein family A (Hsp70) member 9 K04043 dnaK; molecular chaperone DnaK
D 134266 GRPEL2; GrpE like 2, mitochondrial K03687 GRPE; molecular chaperone GrpE
D 80273 GRPEL1; GrpE like 1, mitochondrial K03687 GRPE; molecular chaperone GrpE
D 131118 DNAJC19; DnaJ heat shock protein family (Hsp40) member C19 K09539 DNAJC19; DnaJ homolog subfamily C member 19
D 51025 PAM16; presequence translocase associated motor 16 K17805 PAM16; mitochondrial import inner membrane translocase subunit TIM16
C Other matrix factors
D 23203 PMPCA; peptidase, mitochondrial processing subunit alpha K01412 PMPCA; mitochondrial-processing peptidase subunit alpha [EC:3.4.24.64]
D 9512 PMPCB; peptidase, mitochondrial processing subunit beta K17732 PMPCB; mitochondrial-processing peptidase subunit beta [EC:3.4.24.64]
D 4285 MIPEP; mitochondrial intermediate peptidase K01410 MIPEP; mitochondrial intermediate peptidase [EC:3.4.24.59]
D 3329 HSPD1; heat shock protein family D (Hsp60) member 1 K04077 groEL; chaperonin GroEL [EC:5.6.1.7]
D 3336 HSPE1; heat shock protein family E (Hsp10) member 1 K04078 groES; chaperonin GroES
D 9093 DNAJA3; DnaJ heat shock protein family (Hsp40) member A3 K09504 DNAJA3; DnaJ homolog subfamily A member 3
D 132001 TAMM41; TAM41 mitochondrial translocator assembly and maintenance homolog K17807 TAM41; mitochondrial translocator assembly and maintenance protein 41
D 728489 DNLZ; DNL-type zinc finger K17808 ZIM17; mitochondrial protein import protein ZIM17
D 23474 ETHE1; ETHE1 persulfide dioxygenase K17725 ETHE1; sulfur dioxygenase [EC:1.13.11.18]
D 26073 POLDIP2; DNA polymerase delta interacting protein 2 K17809 POLDIP2; polymerase delta-interacting protein 2
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AMitochondrial quality control factors
B Regulator of mitochondrial biogenesis
C Ubiquitas transcription factors
D 1385 CREB1; cAMP responsive element binding protein 1 K05870 CREB1; cyclic AMP-responsive element-binding protein 1
D 84340 GFM2; GTP dependent ribosome recycling factor mitochondrial 2 K02355 fusA; elongation factor G
D 85476 GFM1; G elongation factor mitochondrial 1 K02355 fusA; elongation factor G
D 6667 SP1; Sp1 transcription factor K04684 SP1; transcription factor Sp1
D 7528 YY1; YY1 transcription factor K09201 YY; transcription factor YY
D 404281 YY2; YY2 transcription factor K09201 YY; transcription factor YY
D 2553 GABPB1; GA binding protein transcription factor subunit beta 1 K09454 GABPB; GA-binding protein transcription factor, beta
D 126626 GABPB2; GA binding protein transcription factor subunit beta 2 K09454 GABPB; GA-binding protein transcription factor, beta
D 7019 TFAM; transcription factor A, mitochondrial K11830 TFAM; transcription factor A, mitochondrial
C Nuclear respiratory factors
D 4899 NRF1; nuclear respiratory factor 1 K11831 NRF1; nuclear respiratory factor 1
D 4780 NFE2L2; NFE2 like bZIP transcription factor 2 K05638 NFE2L2; nuclear factor erythroid 2-related factor 2
D 4490 MT1B; metallothionein 1B K14739 MT1_2; metallothionein 1/2
D 4493 MT1E; metallothionein 1E K14739 MT1_2; metallothionein 1/2
D 4494 MT1F; metallothionein 1F K14739 MT1_2; metallothionein 1/2
D 4495 MT1G; metallothionein 1G K14739 MT1_2; metallothionein 1/2
D 4496 MT1H; metallothionein 1H K14739 MT1_2; metallothionein 1/2
D 4499 MT1M; metallothionein 1M K14739 MT1_2; metallothionein 1/2
D 4501 MT1X; metallothionein 1X K14739 MT1_2; metallothionein 1/2
D 4502 MT2A; metallothionein 2A K14739 MT1_2; metallothionein 1/2
D 4489 MT1A; metallothionein 1A K14739 MT1_2; metallothionein 1/2
D 645745 MT1HL1; metallothionein 1H like 1 K14739 MT1_2; metallothionein 1/2
D 107984936 metallothionein-1E-like K14739 MT1_2; metallothionein 1/2
D 4504 MT3; metallothionein 3 K14740 MT3; metallothionein 3
D 84560 MT4; metallothionein 4 K14741 MT4; metallothionein 4
C Coactivators
D 10891 PPARGC1A; PPARG coactivator 1 alpha K07202 PPARGC1A; alpha peroxisome proliferator-activated receptor gamma coactivator 1-alpha
D 133522 PPARGC1B; PPARG coactivator 1 beta K17962 PPARGC1B; peroxisome proliferator-activated receptor gamma coactivator 1-beta
D 23082 PPRC1; PPARG related coactivator 1 K17963 PPRC1; peroxisome proliferator-activated receptor gamma coactivator-related protein 1
D 23373 CRTC1; CREB regulated transcription coactivator 1 K15309 CRTC1; CREB-regulated transcription coactivator 1
D 1387 CREBBP; CREB binding protein K04498 EP300; E1A/CREB-binding protein [EC:2.3.1.48]
D 2033 EP300; E1A binding protein p300 K04498 EP300; E1A/CREB-binding protein [EC:2.3.1.48]
C Proteins involved in Fe-S cluster biogenesis
D 9054 NFS1; NFS1 cysteine desulfurase K04487 iscS; cysteine desulfurase [EC:2.8.1.7]
D 23479 ISCU; iron-sulfur cluster assembly enzyme K22068 ISCU; iron-sulfur cluster assembly enzyme ISCU, mitochondrial
D 57128 LYRM4; LYR motif containing 4 K22069 LYRM4; LYR motif-containing protein 4
D 2395 FXN; frataxin K19054 FXN; frataxin [EC:1.16.3.1]
D 2230 FDX1; ferredoxin 1 K22070 FDX1; ferredoxin-1, mitochondrial
D 112812 FDX2; ferredoxin 2 K22071 FDX2; ferredoxin-2, mitochondrial
D 2232 FDXR; ferredoxin reductase K18914 FDXR; adrenodoxin-NADP+ reductase [EC:1.18.1.6]
D 3313 HSPA9; heat shock protein family A (Hsp70) member 9 K04043 dnaK; molecular chaperone DnaK
D 150274 HSCB; HscB mitochondrial iron-sulfur cluster cochaperone K04082 hscB; molecular chaperone HscB
D 64374 SIL1; SIL1 nucleotide exchange factor K14001 SIL1; nucleotide exchange factor SIL1
D 51218 GLRX5; glutaredoxin 5 K07390 grxD; monothiol glutaredoxin
D 81689 ISCA1; iron-sulfur cluster assembly 1 K22063 ISCA1; iron-sulfur cluster assembly 1
D 122961 ISCA2; iron-sulfur cluster assembly 2 K22072 ISCA2; iron-sulfur cluster assembly 2
D 200205 IBA57; iron-sulfur cluster assembly factor IBA57 K22073 IBA57; transferase CAF17, mitochondrial [EC:2.1.-.-]
D 27247 NFU1; NFU1 iron-sulfur cluster scaffold K22074 NFU1; NFU1 iron-sulfur cluster scaffold homolog, mitochondrial
D 51027 BOLA1; bolA family member 1 K22066 BOLA1; BolA-like protein 1
D 388962 BOLA3; bolA family member 3 K22075 BOLA3; BolA-like protein 3
D 81855 SFXN3; sideroflexin 3 K23500 SFXN1_3; sideroflexin-1/3
D 94081 SFXN1; sideroflexin 1 K23500 SFXN1_3; sideroflexin-1/3
D 118980 SFXN2; sideroflexin 2 K23501 SFXN2; sideroflexin-2
D 119559 SFXN4; sideroflexin 4 K23502 SFXN4; sideroflexin-4
D 94097 SFXN5; sideroflexin 5 K23503 SFXN5; sideroflexin-5
C Other regulator of mitochondrial biogenesis
D 1374 CPT1A; carnitine palmitoyltransferase 1A K08765 CPT1A; carnitine O-palmitoyltransferase 1, liver isoform [EC:2.3.1.21]
D 1375 CPT1B; carnitine palmitoyltransferase 1B K19523 CPT1B; carnitine O-palmitoyltransferase 1, muscle isoform [EC:2.3.1.21]
D 126129 CPT1C; carnitine palmitoyltransferase 1C K19524 CPT1C; carnitine O-palmitoyltransferase 1, brain isoform [EC:2.3.1.21]
D 55526 DHTKD1; dehydrogenase E1 and transketolase domain containing 1 K15791 DHKTD1; 2-oxoadipate dehydrogenase E1 component [EC:1.2.4.-]
D 26235 FBXL4; F-box and leucine rich repeat protein 4 K10270 FBXL4; F-box and leucine-rich repeat protein 4
D 3054 HCFC1; host cell factor C1 K14966 HCFC1; host cell factor 1
D 29915 HCFC2; host cell factor C2 K27390 HCFC2; host cell factor 2
D 3064 HTT; huntingtin K04533 HD; huntingtin
D 2101 ESRRA; estrogen related receptor alpha K08552 NR3B1; estrogen-related receptor alpha
D 2103 ESRRB; estrogen related receptor beta K08553 NR3B2; estrogen-related receptor beta
D 2104 ESRRG; estrogen related receptor gamma K08554 NR3B3; estrogen-related receptor gamma
D 8204 NRIP1; nuclear receptor interacting protein 1 K17965 NRIP1; nuclear receptor-interacting protein 1
D 5562 PRKAA1; protein kinase AMP-activated catalytic subunit alpha 1 K07198 PRKAA; 5'-AMP-activated protein kinase, catalytic alpha subunit [EC:2.7.11.31]
D 5563 PRKAA2; protein kinase AMP-activated catalytic subunit alpha 2 K07198 PRKAA; 5'-AMP-activated protein kinase, catalytic alpha subunit [EC:2.7.11.31]
D 9361 LONP1; lon peptidase 1, mitochondrial K08675 PRSS15; ATP-dependent Lon protease [EC:3.4.21.53]
D 23411 SIRT1; sirtuin 1 K11411 SIRT1; NAD-dependent protein deacetylase sirtuin 1 [EC:2.3.1.286]
D 84947 SERAC1; serine active site containing 1 K23504 SERAC1; protein SERAC1
D 8409 UXT; ubiquitously expressed prefoldin like chaperone K26537 UXT; protein UXT
D 84808 PERM1; PPARGC1 and ESRR induced regulator, muscle 1 K26738 PERM1; PGC-1 and ERR-induced regulator in muscle protein 1
D 102724023 GATD3B; glutamine amidotransferase class 1 domain containing 3B K27835 GATD3; glutamine amidotransferase-like class 1 domain-containing protein 3, mitochondrial
D 8209 GATD3; glutamine amidotransferase class 1 domain containing 3 K27835 GATD3; glutamine amidotransferase-like class 1 domain-containing protein 3, mitochondrial
B Mitochondrial respiratory chain complex assembly factors
C Complex-I assembly factors
D 51103 NDUFAF1; NADH:ubiquinone oxidoreductase complex assembly factor 1 K18159 NDUFAF1; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex assembly factor 1
D 91942 NDUFAF2; NADH:ubiquinone oxidoreductase complex assembly factor 2 K18160 NDUFAF2; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex assembly factor 2
D 25915 NDUFAF3; NADH:ubiquinone oxidoreductase complex assembly factor 3 K09008 NDUFAF3; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex assembly factor 3
D 29078 NDUFAF4; NADH:ubiquinone oxidoreductase complex assembly factor 4 K18161 NDUFAF4; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex assembly factor 4
D 79133 NDUFAF5; NADH:ubiquinone oxidoreductase complex assembly factor 5 K18162 NDUFAF5; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex assembly factor 5 [EC:2.1.1.-]
D 137682 NDUFAF6; NADH:ubiquinone oxidoreductase complex assembly factor 6 K18163 NDUFAF6; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex assembly factor 6
D 55471 NDUFAF7; NADH:ubiquinone oxidoreductase complex assembly factor 7 K18164 NDUFAF7; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex assembly factor 7
D 284184 NDUFAF8; NADH:ubiquinone oxidoreductase complex assembly factor 8 K24726 NDUFAF8; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex assembly factor 8
D 55863 TMEM126B; transmembrane protein 126B K18165 TMEM126B; complex I assembly factor TMEM126B
D 51295 ECSIT; ECSIT signaling integrator K04405 ECSIT; evolutionarily conserved signaling intermediate in Toll pathway
D 28976 ACAD9; acyl-CoA dehydrogenase family member 9 K15980 ACAD9; acyl-CoA dehydrogenase family member 9 [EC:1.3.99.-]
D 80224 NUBPL; NUBP iron-sulfur cluster assembly factor, mitochondrial K03593 mrp; ATP-binding protein involved in chromosome partitioning
D 55572 FOXRED1; FAD dependent oxidoreductase domain containing 1 K18166 FOXRED1; FAD-dependent oxidoreductase domain-containing protein 1
D 84816 RTN4IP1; reticulon 4 interacting protein 1 K23164 RTN4IP1; reticulon-4-interacting protein 1, mitochondrial
D 51300 TIMMDC1; translocase of inner mitochondrial membrane domain containing 1 K23505 TIMMDC1; complex I assembly factor TIMMDC1
C Complex-II assembly factors
D 644096 SDHAF1; succinate dehydrogenase complex assembly factor 1 K18167 SDHAF1; succinate dehydrogenase assembly factor 1
D 54949 SDHAF2; succinate dehydrogenase complex assembly factor 2 K18168 SDHAF2; succinate dehydrogenase assembly factor 2
C Complex-III assembly factors
D 617 BCS1L; BCS1 homolog, ubiquinol-cytochrome c reductase complex chaperone K08900 BCS1; mitochondrial chaperone BCS1
D 54902 TTC19; tetratricopeptide repeat domain 19 K18169 TTC19; tetratricopeptide repeat protein 19, mitochondrial
D 90624 LYRM7; LYR motif containing 7 K18170 LYRM7; complex III assembly factor LYRM7
D 55245 UQCC1; ubiquinol-cytochrome c reductase complex assembly factor 1 K17662 CBP3; cytochrome b pre-mRNA-processing protein 3
D 84300 UQCC2; ubiquinol-cytochrome c reductase complex assembly factor 2 K17682 MNF1; mitochondrial nucleoid factor 1
D 790955 UQCC3; ubiquinol-cytochrome c reductase complex assembly factor 3 K23165 UQCC3; ubiquinol-cytochrome-c reductase complex assembly factor 3
D 283951 UQCC4; ubiquinol-cytochrome c reductase complex assembly factor 4 K27339 UQCC4; ubiquinol-cytochrome c reductase complex assembly factor 4
D 440957 UQCC5; ubiquinol-cytochrome c reductase complex assembly factor 5 K27340 UQCC5; ubiquinol-cytochrome c reductase complex assembly factor 5
D 728568 UQCC6; ubiquinol-cytochrome c reductase complex assembly factor 6 K27341 UQCC6; ubiquinol-cytochrome c reductase complex assembly factor 6
D 647087 STMP1; short transmembrane mitochondrial protein 1 K27319 STMP1; short transmembrane mitochondrial protein 1
C Complex-IV assembly factors
D 152100 CMC1; C-X9-C motif containing 1 K18171 CMC1; COX assembly mitochondrial protein 1
D 56942 CMC2; C-X9-C motif containing 2 K18172 CMC2; COX assembly mitochondrial protein 2
D 55744 COA1; cytochrome c oxidase assembly factor 1 K18173 COA1; cytochrome c oxidase assembly factor 1
D 28958 COA3; cytochrome c oxidase assembly factor 3 K18175 CCDC56; cytochrome c oxidase assembly factor 3, animal type
D 51287 COA4; cytochrome c oxidase assembly factor 4 homolog K18177 COA4; cytochrome c oxidase assembly factor 4
D 493753 COA5; cytochrome c oxidase assembly factor 5 K18178 COA5; cytochrome c oxidase assembly factor 5
D 388753 COA6; cytochrome c oxidase assembly factor 6 K18179 COA6; cytochrome c oxidase assembly factor 6
D 65260 COA7; cytochrome c oxidase assembly factor 7 K18180 COA7; cytochrome c oxidase assembly factor 7
D 1352 COX10; cytochrome c oxidase assembly factor heme A:farnesyltransferase COX10 K02257 COX10; heme o synthase [EC:2.5.1.141]
D 1353 COX11; cytochrome c oxidase copper chaperone COX11 K02258 COX11; cytochrome c oxidase assembly protein subunit 11
D 84987 COX14; cytochrome c oxidase assembly factor COX14 K18181 COX14; cytochrome c oxidase assembly factor 14
D 1355 COX15; cytochrome c oxidase assembly homolog COX15 K02259 COX15; heme a synthase [EC:1.17.99.9]
D 51241 COX16; cytochrome c oxidase assembly factor COX16 K18182 COX16; cytochrome c oxidase assembly protein subunit 16
D 10063 COX17; cytochrome c oxidase copper chaperone COX17 K02260 COX17; cytochrome c oxidase assembly protein subunit 17
D 285521 COX18; cytochrome c oxidase assembly factor COX18 K17797 COX18; mitochondrial inner membrane protein COX18
D 90639 COX19; cytochrome c oxidase assembly factor COX19 K18183 COX19; cytochrome c oxidase assembly protein subunit 19
D 116228 COX20; cytochrome c oxidase assembly factor COX20 K18184 COX20; cytochrome c oxidase assembly protein subunit 20
D 118490 MSS51; MSS51 mitochondrial translational activator K17656 MSS51; mitochondrial splicing suppressor protein 51
D 100131801 PET100; PET100 cytochrome c oxidase chaperone K18186 PET100; protein PET100, animal type
D 100303755 PET117; PET117 cytochrome c oxidase chaperone K18188 PET117; protein PET117
D 5018 OXA1L; OXA1L mitochondrial inner membrane protein K03217 yidC; YidC/Oxa1 family membrane protein insertase
D 6834 SURF1; SURF1 cytochrome c oxidase assembly factor K14998 SURF1; surfeit locus 1 family protein
D 6341 SCO1; synthesis of cytochrome C oxidase 1 K07152 SCO1; protein SCO1
D 9997 SCO2; synthesis of cytochrome C oxidase 2 K23755 SCO2; protein SCO2
D 51204 TACO1; translational activator of cytochrome c oxidase I K18189 TACO1; translational activator of cytochrome c oxidase 1
D 10128 LRPPRC; leucine rich pentatricopeptide repeat containing K17964 LRPPRC; leucine-rich PPR motif-containing protein, mitochondrial
D 84334 COA8; cytochrome c oxidase assembly factor 8 K23506 APOPT1; apoptogenic protein 1
D 647087 STMP1; short transmembrane mitochondrial protein 1 K27319 STMP1; short transmembrane mitochondrial protein 1
D 440957 UQCC5; ubiquinol-cytochrome c reductase complex assembly factor 5 K27340 UQCC5; ubiquinol-cytochrome c reductase complex assembly factor 5
D 79064 TMEM223; transmembrane protein 223 K27342 TMEM223; transmembrane protein 223
C Complex-V assembly factors
D 64756 ATPAF1; ATP synthase mitochondrial F1 complex assembly factor 1 K07555 ATPeAF1; ATP synthase mitochondrial F1 complex assembly factor 1
D 91647 ATPAF2; ATP synthase mitochondrial F1 complex assembly factor 2 K07556 ATPeAF2; ATP synthase mitochondrial F1 complex assembly factor 2
D 54968 TMEM70; transmembrane protein 70 K17966 TMEM70; transmembrane protein 70, mitochondrial
D 9556 ATP5MJ; ATP synthase membrane subunit j K18193 MP68; 6.8 kDa mitochondrial proteolipid
D 112268080 ATP synthase subunit ATP5MJ, mitochondrial-like K18193 MP68; 6.8 kDa mitochondrial proteolipid
D 84833 ATP5MK; ATP synthase membrane subunit k K18194 USMG5; up-regulated during skeletal muscle growth protein 5
B Mitochondrial dynamics
C Fission and Fusion factors
D 55669 MFN1; mitofusin 1 K21356 MFN1; mitofusin 1 [EC:3.6.5.-]
D 9927 MFN2; mitofusin 2 K06030 MFN2; mitofusin 2 [EC:3.6.5.-]
D 4976 OPA1; OPA1 mitochondrial dynamin like GTPase K17079 OPA1; optic atrophy protein 1 [EC:3.6.5.5]
D 80207 OPA3; outer mitochondrial membrane lipid metabolism regulator OPA3 K23166 OPA3; optic atrophy 3 protein
D 51499 TRIAP1; TP53 regulated inhibitor of apoptosis 1 K17968 TRIAP1; TRIAP1/MDM35 family protein
D 55486 PARL; presenilin associated rhomboid like K09650 PARL; rhomboid-like protein [EC:3.4.21.105]
D 578 BAK1; BCL2 antagonist/killer 1 K14021 BAK; Bcl-2 homologous antagonist/killer
D 581 BAX; BCL2 associated X, apoptosis regulator K02159 BAX; apoptosis regulator BAX
D 10059 DNM1L; dynamin 1 like K17065 DNM1L; dynamin 1-like protein [EC:3.6.5.5]
D 51024 FIS1; fission, mitochondrial 1 K17969 FIS1; mitochondrial fission 1 protein
D 6612 SUMO3; small ubiquitin like modifier 3 K12160 SUMO; small ubiquitin-related modifier
D 6613 SUMO2; small ubiquitin like modifier 2 K12160 SUMO; small ubiquitin-related modifier
D 7341 SUMO1; small ubiquitin like modifier 1 K12160 SUMO; small ubiquitin-related modifier
D 387082 SUMO4; small ubiquitin like modifier 4 K12160 SUMO; small ubiquitin-related modifier
D 51100 SH3GLB1; SH3 domain containing GRB2 like, endophilin B1 K11248 SH3GLB1; endophilin-B1
D 23787 MTCH1; mitochondrial carrier 1 K17885 MTCH; mitochondrial carrier
D 23788 MTCH2; mitochondrial carrier 2 K17885 MTCH; mitochondrial carrier
D 56947 MFF; mitochondrial fission factor K22076 MFF; mitochondrial fission factor
D 54332 GDAP1; ganglioside induced differentiation associated protein 1 K22077 GDAP1; ganglioside-induced differentiation-associated protein 1
D 78997 GDAP1L1; ganglioside induced differentiation associated protein 1 like 1 K22077 GDAP1; ganglioside-induced differentiation-associated protein 1
D 10493 VAT1; vesicle amine transport 1 K23167 VAT1; synaptic vesicle membrane protein VAT-1
D 125170 MIEF2; mitochondrial elongation factor 2 K23507 MIEF1_2; mitochondrial dynamics protein MID51/49
D 54471 MIEF1; mitochondrial elongation factor 1 K23507 MIEF1_2; mitochondrial dynamics protein MID51/49
D 201164 PLD6; phospholipase D family member 6 K16862 PLD6; mitochondrial cardiolipin hydrolase [EC:3.1.4.-]
D 374986 MIGA1; mitoguardin 1 K27289 MIGA; mitoguardin
D 84895 MIGA2; mitoguardin 2 K27289 MIGA; mitoguardin
C Actin cytoskeleton organization
D 71 ACTG1; actin gamma 1 K05692 ACTB_G1; actin beta/gamma 1
D 60 ACTB; actin beta K05692 ACTB_G1; actin beta/gamma 1
D 51126 NAA20; N-alpha-acetyltransferase 20, NatB catalytic subunit K17972 NAA20; N-terminal acetyltransferase B complex catalytic subunit [EC:2.3.1.254]
D 80018 NAA25; N-alpha-acetyltransferase 25, NatB auxiliary subunit K17973 NAA25; N-terminal acetyltransferase B complex non-catalytic subunit
C Mitochondrial movement
D 5245 PHB1; prohibitin 1 K17080 PHB1; prohibitin 1
D 11331 PHB2; prohibitin 2 K17081 PHB2; prohibitin 2
D 3895 KTN1; kinectin 1 K17975 KTN1; kinectin
C Retention of mitochondria
D 10059 DNM1L; dynamin 1 like K17065 DNM1L; dynamin 1-like protein [EC:3.6.5.5]
D 4644 MYO5A; myosin VA K10357 MYO5; myosin V
D 4645 MYO5B; myosin VB K10357 MYO5; myosin V
D 55930 MYO5C; myosin VC K10357 MYO5; myosin V
D 331 XIAP; X-linked inhibitor of apoptosis K04725 XIAP; E3 ubiquitin-protein ligase XIAP [EC:2.3.2.27]
D 56616 DIABLO; diablo IAP-binding mitochondrial protein K10522 DIABLO; diablo
C Morphogenesis factors
D 83891 SNX25; sorting nexin 25 K17887 SNX25; sorting nexin-25
D 55288 RHOT1; ras homolog family member T1 K07870 RHOT1; mitochondrial Rho GTPase 1 [EC:3.6.5.-]
D 10989 IMMT; inner membrane mitochondrial protein K17785 IMMT; MICOS complex subunit MIC60
D 51537 MTFP1; mitochondrial fission process 1 K17981 MTFP1; mitochondrial fission process protein 1
D 7818 DAP3; death associated protein 3 K17408 DAP3; small subunit ribosomal protein S29
D 4734 NEDD4; NEDD4 E3 ubiquitin protein ligase K10591 NEDD4; E3 ubiquitin-protein ligase NEDD4 [EC:2.3.2.26]
D 55074 OXR1; oxidation resistance 1 K25437 OXR1; oxidation resistance protein 1
D 55154 MSTO1; misato mitochondrial distribution and morphology regulator 1 K27320 MSTO1; protein misato
B Mitophagy factors
C Autophagy-related proteins
D 23130 ATG2A; autophagy related 2A K17906 ATG2; autophagy-related protein 2
D 55102 ATG2B; autophagy related 2B K17906 ATG2; autophagy-related protein 2
D 64422 ATG3; autophagy related 3 K08343 ATG3; ubiquitin-like-conjugating enzyme ATG3
D 115201 ATG4A; autophagy related 4A cysteine peptidase K08342 ATG4; cysteine protease ATG4 [EC:3.4.22.-]
D 23192 ATG4B; autophagy related 4B cysteine peptidase K08342 ATG4; cysteine protease ATG4 [EC:3.4.22.-]
D 84938 ATG4C; autophagy related 4C cysteine peptidase K08342 ATG4; cysteine protease ATG4 [EC:3.4.22.-]
D 84971 ATG4D; autophagy related 4D cysteine peptidase K08342 ATG4; cysteine protease ATG4 [EC:3.4.22.-]
D 9474 ATG5; autophagy related 5 K08339 ATG5; autophagy-related protein 5
D 8678 BECN1; beclin 1 K08334 BECN; beclin
D 441925 BECN2; beclin 2 K08334 BECN; beclin
D 10533 ATG7; autophagy related 7 K08337 ATG7; ubiquitin-like modifier-activating enzyme ATG7
D 11337 GABARAP; GABA type A receptor-associated protein K08341 GABARAP; GABA(A) receptor-associated protein
D 23710 GABARAPL1; GABA type A receptor associated protein like 1 K08341 GABARAP; GABA(A) receptor-associated protein
D 11345 GABARAPL2; GABA type A receptor associated protein like 2 K08341 GABARAP; GABA(A) receptor-associated protein
D 285973 ATG9B; autophagy related 9B K17907 ATG9; autophagy-related protein 9
D 79065 ATG9A; autophagy related 9A K17907 ATG9; autophagy-related protein 9
D 83734 ATG10; autophagy related 10 K17888 ATG10L; ubiquitin-like-conjugating enzyme ATG10
D 9140 ATG12; autophagy related 12 K08336 ATG12; ubiquitin-like protein ATG12
D 22863 ATG14; autophagy related 14 K17889 ATG14L; beclin 1-associated autophagy-related key regulator
D 55054 ATG16L1; autophagy related 16 like 1 K17890 ATG16L1; autophagy-related protein 16-1
D 89849 ATG16L2; autophagy related 16 like 2 K20868 ATG16L2; autophagy-related protein 16-2
D 26100 WIPI2; WD repeat domain, phosphoinositide interacting 2 K17908 WIPI1_2; autophagy-related protein 18
D 55062 WIPI1; WD repeat domain, phosphoinositide interacting 1 K17908 WIPI1_2; autophagy-related protein 18
C Parkin-dependent mechanism factors
D 5071 PRKN; parkin RBR E3 ubiquitin protein ligase K04556 PARK2; parkin [EC:2.3.2.31]
D 10193 RNF41; ring finger protein 41 K11981 RNF41; E3 ubiquitin-protein ligase NRDP1 [EC:2.3.2.27]
D 23274 CLEC16A; C-type lectin domain containing 16A K19513 CLEC16A; protein CLEC16A
D 65018 PINK1; PTEN induced kinase 1 K05688 PINK1; PTEN induced putative kinase 1 [EC:2.7.11.1]
D 55486 PARL; presenilin associated rhomboid like K09650 PARL; rhomboid-like protein [EC:3.4.21.105]
D 8878 SQSTM1; sequestosome 1 K14381 SQSTM1; sequestosome 1
D 8887 TAX1BP1; Tax1 binding protein 1 K21347 TAX1BP1; Tax1-binding protein 1
D 10241 CALCOCO2; calcium binding and coiled-coil domain 2 K21348 CALCOCO2; calcium-binding and coiled-coil domain-containing protein 2
D 55626 AMBRA1; autophagy and beclin 1 regulator 1 K17985 AMBRA1; activating molecule in BECN1-regulated autophagy protein 1
C Parkin-independent mechanism factors
D 9776 ATG13; autophagy related 13 K08331 ATG13; autophagy-related protein 13
D 8408 ULK1; unc-51 like autophagy activating kinase 1 K21357 ULK1; serine/threonine-protein kinase ULK1 [EC:2.7.11.1]
D 9706 ULK2; unc-51 like autophagy activating kinase 2 K08269 ULK2; serine/threonine-protein kinase ULK2 [EC:2.7.11.1]
D 9821 RB1CC1; RB1 inducible coiled-coil 1 K17589 RB1CC1; RB1-inducible coiled-coil protein 1
D 3320 HSP90AA1; heat shock protein 90 alpha family class A member 1 K04079 HSP90A; molecular chaperone HtpG
D 3326 HSP90AB1; heat shock protein 90 alpha family class B member 1 K04079 HSP90A; molecular chaperone HtpG
D 11140 CDC37; cell division cycle 37, HSP90 cochaperone K09554 CDC37; cell division cycle protein 37
D 57154 SMURF1; SMAD specific E3 ubiquitin protein ligase 1 K04678 SMURF; E3 ubiquitin ligase SMURF1/2 [EC:2.3.2.26]
D 64750 SMURF2; SMAD specific E3 ubiquitin protein ligase 2 K04678 SMURF; E3 ubiquitin ligase SMURF1/2 [EC:2.3.2.26]
D 665 BNIP3L; BCL2 interacting protein 3 like K15465 BNIP3L; BCL2/adenovirus E1B 19 kDa protein-interacting protein 3-like
D 139341 FUNDC1; FUN14 domain containing 1 K17986 FUNDC1; FUN14 domain-containing protein 1
C Other mitophagy factors
D 5562 PRKAA1; protein kinase AMP-activated catalytic subunit alpha 1 K07198 PRKAA; 5'-AMP-activated protein kinase, catalytic alpha subunit [EC:2.7.11.31]
D 5563 PRKAA2; protein kinase AMP-activated catalytic subunit alpha 2 K07198 PRKAA; 5'-AMP-activated protein kinase, catalytic alpha subunit [EC:2.7.11.31]
D 440738 MAP1LC3C; microtubule associated protein 1 light chain 3 gamma K10435 MAP1LC; microtubule-associated protein 1 light chain
D 81631 MAP1LC3B; microtubule associated protein 1 light chain 3 beta K10435 MAP1LC; microtubule-associated protein 1 light chain
D 84557 MAP1LC3A; microtubule associated protein 1 light chain 3 alpha K10435 MAP1LC; microtubule-associated protein 1 light chain
D 643246 MAP1LC3B2; microtubule associated protein 1 light chain 3 beta 2 K10435 MAP1LC; microtubule-associated protein 1 light chain
D 392288 MAP1LC3BP1; microtubule associated protein 1 light chain 3 beta pseudogene 1 K10435 MAP1LC; microtubule-associated protein 1 light chain
D 4077 NBR1; NBR1 autophagy cargo receptor K17987 NBR1; next to BRCA1 gene 1 protein
D 25851 TECPR1; tectonin beta-propeller repeat containing 1 K17988 TECPR1; tectonin beta-propeller repeat-containing protein 1
D 53349 ZFYVE1; zinc finger FYVE-type containing 1 K17603 ZFYVE1; zinc finger FYVE domain-containing protein 1
D 80347 COASY; Coenzyme A synthase K02318 COASY; phosphopantetheine adenylyltransferase / dephospho-CoA kinase [EC:2.7.7.3 2.7.1.24]
D 8398 PLA2G6; phospholipase A2 group VI K16343 PLA2G6; calcium-independent phospholipase A2 [EC:3.1.1.4]
D 83636 C19orf12; chromosome 19 open reading frame 12 K23168 MPAN; protein MPAN
D 65991 FUNDC2; FUN14 domain containing 2 K24308 FUNDC2; FUN14 domain-containing protein 2
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#[ BRITE | KEGG2 | KEGG ]
#Last updated: August 9, 2024