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Mitochondrial Biogenesis - Ornithorhynchus anatinus (platypus)
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AMitochondrial DNA transcription, translation, and replication factors
B Mitochondrial DNA-encoded proteins
C Mitochondrial respiratory chain complex I
D 808708 ND1; NADH dehydrogenase subunit 1 K03878 ND1; NADH-ubiquinone oxidoreductase chain 1 [EC:7.1.1.2]
D 808700 ND2; NADH dehydrogenase subunit 2 K03879 ND2; NADH-ubiquinone oxidoreductase chain 2 [EC:7.1.1.2]
D 808710 ND3; NADH dehydrogenase subunit 3 K03880 ND3; NADH-ubiquinone oxidoreductase chain 3 [EC:7.1.1.2]
D 808699 ND4; NADH dehydrogenase subunit 4 K03881 ND4; NADH-ubiquinone oxidoreductase chain 4 [EC:7.1.1.2]
D 808698 ND4L; NADH dehydrogenase subunit 4L K03882 ND4L; NADH-ubiquinone oxidoreductase chain 4L [EC:7.1.1.2]
D 808701 ND5; NADH dehydrogenase subunit 5 K03883 ND5; NADH-ubiquinone oxidoreductase chain 5 [EC:7.1.1.2]
D 808706 ND6; NADH dehydrogenase subunit 6 K03884 ND6; NADH-ubiquinone oxidoreductase chain 6 [EC:7.1.1.2]
C Mitochondrial respiratory chain complex III
D 808703 CYTB; cytochrome b K00412 CYTB; ubiquinol-cytochrome c reductase cytochrome b subunit
C Cytochrome c oxidase
D 808704 COX1; cytochrome c oxidase subunit I K02256 COX1; cytochrome c oxidase subunit 1 [EC:7.1.1.9]
D 808709 COX2; cytochrome c oxidase subunit II K02261 COX2; cytochrome c oxidase subunit 2
D 808707 COX3; cytochrome c oxidase subunit III K02262 COX3; cytochrome c oxidase subunit 3
C F-type ATPase
D 808702 ATP8; ATP synthase F0 subunit 8 K02125 ATPeF08; F-type H+-transporting ATPase subunit 8
D 808705 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 103166531 POLRMT; DNA-directed RNA polymerase, mitochondrial K10908 POLRMT; DNA-directed RNA polymerase, mitochondrial [EC:2.7.7.6]
C Mitochondrial transcription factors
D 100076559 transcription factor A, mitochondrial-like K11830 TFAM; transcription factor A, mitochondrial
D 100076636 transcription factor A, mitochondrial-like K11830 TFAM; transcription factor A, mitochondrial
D 100079786 TFB1M; dimethyladenosine transferase 1, mitochondrial isoform X1 K15266 TFB1M; dimethyladenosine transferase 1, mitochondrial [EC:2.1.1.-]
D 100082724 TFB2M; dimethyladenosine transferase 2, mitochondrial isoform X1 K17653 TFB2M; dimethyladenosine transferase 2, mitochondrial [EC:2.1.1.-]
C Mitochondrial translation factors
D 100079175 MTIF2; translation initiation factor IF-2, mitochondrial isoform X1 K02519 infB; translation initiation factor IF-2
D 100090314 MTIF3; translation initiation factor IF-3, mitochondrial K02520 infC; translation initiation factor IF-3
D 114814664 TSFM; elongation factor Ts, mitochondrial K02357 tsf; elongation factor Ts
D 100090114 TUFM; elongation factor Tu, mitochondrial isoform X1 K02358 tuf; elongation factor Tu
D 100082870 GFM2; ribosome-releasing factor 2, mitochondrial K02355 fusA; elongation factor G
D 100086302 GFM1; elongation factor G, mitochondrial K02355 fusA; elongation factor G
D 100073368 MTERF3; transcription termination factor 3, mitochondrial K15031 MTERF; mitochondrial transcription termination factor family protein
D 100077287 MTERF2; transcription termination factor 2, mitochondrial K15031 MTERF; mitochondrial transcription termination factor family protein
D 100082754 MTERF4; transcription termination factor 4, mitochondrial isoform X1 K15031 MTERF; mitochondrial transcription termination factor family protein
D 100089374 MTERF1; transcription termination factor 1, mitochondrial K15031 MTERF; mitochondrial transcription termination factor family protein
D 100085632 GUF1; translation factor GUF1, mitochondrial K21594 GUF1; translation factor GUF1, mitochondrial [EC:3.6.5.-]
C RNase MRP
D 100093547 POP1; ribonucleases P/MRP protein subunit POP1 K01164 POP1; ribonuclease P/MRP protein subunit POP1 [EC:3.1.26.5]
D 100075545 POP4; ribonuclease P protein subunit p29 K03538 POP4; ribonuclease P protein subunit POP4 [EC:3.1.26.5]
D 100089087 POP5; ribonuclease P/MRP protein subunit POP5 K03537 POP5; ribonuclease P/MRP protein subunit POP5 [EC:3.1.26.5]
D 100083837 RPP30; ribonuclease P protein subunit p30 K03539 RPP1; ribonuclease P/MRP protein subunit RPP1 [EC:3.1.26.5]
D 100088536 POP7; ribonuclease P protein subunit p20 K14527 RPP20; ribonuclease P/MRP protein subunit RPP20 [EC:3.1.26.5]
D 100088902 RPP25L; ribonuclease P protein subunit p25-like protein K14525 RPP25; ribonucleases P/MRP protein subunit RPP25 [EC:3.1.26.5]
D 114812096 RPP25; ribonuclease P protein subunit p25 K14525 RPP25; ribonucleases P/MRP protein subunit RPP25 [EC:3.1.26.5]
D 100681328 RPP38; ribonuclease P protein subunit p38 K14523 RPP38; ribonucleases P/MRP protein subunit RPP38 [EC:3.1.26.5]
D 100076333 RPP40; ribonuclease P protein subunit p40 K14530 RPP40; ribonucleases P/MRP protein subunit RPP40 [EC:3.1.26.5]
C Mitochondrial ribonuclease P
D 100089544 TRMT10C; tRNA methyltransferase 10 homolog C K17654 TRMT10C; mitochondrial ribonuclease P protein 1 [EC:2.1.1.-]
D 100081790 HSD17B10; 3-hydroxyacyl-CoA dehydrogenase type-2 K08683 HSD17B10; 3-hydroxyacyl-CoA dehydrogenase / 3-hydroxy-2-methylbutyryl-CoA dehydrogenase [EC:1.1.1.35 1.1.1.178]
D 100081197 PRORP; mitochondrial ribonuclease P catalytic subunit K17655 MRPP3; mitochondrial ribonuclease P protein 3
C Other mitochondrial DNA transcription and translation factors
D 103171428 MSS51; putative protein MSS51 homolog, mitochondrial isoform X1 K17656 MSS51; mitochondrial splicing suppressor protein 51
D 100080330 TEFM; transcription elongation factor, mitochondrial K17658 TEFM; transcription elongation factor, mitochondrial
D 103165473 PTCD1; pentatricopeptide repeat-containing protein 1, mitochondrial isoform X1 K17710 PTCD1; pentatricopeptide repeat domain-containing protein 1
D 100084364 PTCD2; pentatricopeptide repeat-containing protein 2, mitochondrial K17711 PTCD2; pentatricopeptide repeat domain-containing protein 2
D 100081180 PTCD3; pentatricopeptide repeat domain-containing protein 3, mitochondrial K17659 PTCD3; pentatricopeptide repeat domain-containing protein 3
D 100087069 ATP-dependent RNA helicase DHX30 K13185 DHX30; ATP-dependent RNA helicase DHX30 [EC:5.6.2.6]
D 100681421 DHX30; ATP-dependent RNA helicase DHX30 isoform X1 K13185 DHX30; ATP-dependent RNA helicase DHX30 [EC:5.6.2.6]
D 114806165 ubiquinol-cytochrome-c reductase complex assembly factor 1 K17662 CBP3; cytochrome b pre-mRNA-processing protein 3
D 100080332 AFG1L; AFG1-like ATPase isoform X1 K18798 AFG1; peroxisome-assembly ATPase [EC:3.6.4.7]
D 100088941 ATP23; mitochondrial inner membrane protease ATP23 homolog K18156 ATP23; mitochondrial inner membrane protease ATP23 [EC:3.4.24.-]
D 100085419 SUPV3L1; ATP-dependent RNA helicase SUPV3L1, mitochondrial K17675 SUPV3L1; ATP-dependent RNA helicase SUPV3L1/SUV3 [EC:5.6.2.6]
D 100087422 LACTB2; endoribonuclease LACTB2 K16639 LACTB2; endoribonuclease LACTB2 [EC:3.1.27.-]
D 100080434 SLC25A5; ADP/ATP translocase 2 K05863 SLC25A4S; solute carrier family 25 (mitochondrial adenine nucleotide translocator), member 4/5/6/31
D 100087351 SLC25A4; ADP/ATP translocase 1 K05863 SLC25A4S; solute carrier family 25 (mitochondrial adenine nucleotide translocator), member 4/5/6/31
D 114812883 SLC25A31; ADP/ATP translocase 4 K05863 SLC25A4S; solute carrier family 25 (mitochondrial adenine nucleotide translocator), member 4/5/6/31
D 100075853 SLC25A37; mitoferrin-1 isoform X2 K15113 SLC25A28_37; solute carrier family 25 (mitochondrial iron transporter), member 28/37
D 100087876 SLC25A28; mitoferrin-2 isoform X1 K15113 SLC25A28_37; solute carrier family 25 (mitochondrial iron transporter), member 28/37
D 100083116 MALSU1; mitochondrial assembly of ribosomal large subunit protein 1 K25803 MALSU1; mitochondrial assembly of ribosomal large subunit protein 1
D 100087481 ERAL1; GTPase Era, mitochondrial K03595 era; GTPase
D 100084390 YARS2; tyrosine--tRNA ligase, mitochondrial K01866 YARS; tyrosyl-tRNA synthetase [EC:6.1.1.1]
D 103169142 YARS1; tyrosine--tRNA ligase, cytoplasmic K01866 YARS; tyrosyl-tRNA synthetase [EC:6.1.1.1]
D 100081428 LARS1; leucine--tRNA ligase, cytoplasmic isoform X1 K01869 LARS; leucyl-tRNA synthetase [EC:6.1.1.4]
D 100082885 LARS2; probable leucine--tRNA ligase, mitochondrial isoform X1 K01869 LARS; leucyl-tRNA synthetase [EC:6.1.1.4]
D 100084784 DARS2; aspartate--tRNA ligase, mitochondrial K01876 DARS2; aspartyl-tRNA synthetase [EC:6.1.1.12]
D 100092722 GARS1; glycine--tRNA ligase K01880 GARS; glycyl-tRNA synthetase [EC:6.1.1.14]
D 100078336 RARS1; arginine--tRNA ligase, cytoplasmic K01887 RARS; arginyl-tRNA synthetase [EC:6.1.1.19]
D 100082749 RARS2; probable arginine--tRNA ligase, mitochondrial isoform X1 K01887 RARS; arginyl-tRNA synthetase [EC:6.1.1.19]
D 100081215 QRSL1; glutamyl-tRNA(Gln) amidotransferase subunit A, mitochondrial isoform X1 K02433 gatA; aspartyl-tRNA(Asn)/glutamyl-tRNA(Gln) amidotransferase subunit A [EC:6.3.5.6 6.3.5.7]
D 100080760 GATB; glutamyl-tRNA(Gln) amidotransferase subunit B, mitochondrial K02434 gatB; aspartyl-tRNA(Asn)/glutamyl-tRNA(Gln) amidotransferase subunit B [EC:6.3.5.6 6.3.5.7]
D 100076743 glutamyl-tRNA(Gln) amidotransferase subunit C, mitochondrial isoform X1 K02435 gatC; aspartyl-tRNA(Asn)/glutamyl-tRNA(Gln) amidotransferase subunit C [EC:6.3.5.6 6.3.5.7]
D 114809046 glutamyl-tRNA(Gln) amidotransferase subunit C, mitochondrial-like K02435 gatC; aspartyl-tRNA(Asn)/glutamyl-tRNA(Gln) amidotransferase subunit C [EC:6.3.5.6 6.3.5.7]
D 100076255 poly(A) RNA polymerase, mitochondrial isoform X1 K18060 MTPAP; poly(A) RNA polymerase, mitochondrial [EC:2.7.7.19]
D 100080200 PDE12; 2',5'-phosphodiesterase 12 K19612 PDE12; 2',5'-phosphodiesterase [EC:3.1.13.4 3.1.4.-]
D 114806469 LOW QUALITY PROTEIN: 2',5'-phosphodiesterase 12-like K19612 PDE12; 2',5'-phosphodiesterase [EC:3.1.13.4 3.1.4.-]
D 100084905 MTG1; mitochondrial ribosome-associated GTPase 1 K19828 MTG1; mitochondrial GTPase 1
D 100088774 NOA1; nitric oxide-associated protein 1 K19832 NOA1; nitric oxide-associated protein 1
D 100089123 NSUN3; tRNA (cytosine(34)-C(5))-methyltransferase, mitochondrial isoform X1 K21969 NSUN3; tRNA (cytosine34-C5)-methyltransferase [EC:2.1.1.-]
D 100089271 NSUN4; 5-methylcytosine rRNA methyltransferase NSUN4 isoform X1 K21970 NSUN4; 5-methylcytosine rRNA methyltransferase NSUN4 [EC:2.1.1.-]
D 100075205 RPUSD3; mitochondrial mRNA pseudouridine synthase RPUSD3 isoform X1 K22537 RPUSD3; mitochondrial RNA pseudouridine synthase RPUSD3 [EC:5.4.99.-]
D 100088594 RPUSD4; mitochondrial RNA pseudouridine synthase RPUSD4 K22538 RPUSD4; mitochondrial RNA pseudouridine synthase RPUSD4 [EC:5.4.99.-]
D 114817883 RCC1L; RCC1-like G exchanging factor-like protein K23495 RCC1L; RCC1-like G exchanging factor-like protein
D 100091664 TRUB2; mitochondrial mRNA pseudouridine synthase TRUB2 isoform X1 K23497 TRUB2; mitochondrial mRNA pseudouridine synthase TRUB2 [EC:5.4.99.-]
D 100083457 FASTKD1; LOW QUALITY PROTEIN: FAST kinase domain-containing protein 1, mitochondrial K27754 FASTKD1; FAST kinase domain-containing protein 1
D 100076729 FASTKD2; FAST kinase domain-containing protein 2, mitochondrial isoform X1 K18190 FASTKD2; FAST kinase domain-containing protein 2
D 100085203 TBRG4; FAST kinase domain-containing protein 4 K27756 TBRG4; FAST kinase domain-containing protein 4
D 103167260 FASTKD5; FAST kinase domain-containing protein 5, mitochondrial K27757 FASTKD5; FAST kinase domain-containing protein 5
D 100075611 MTRFR; probable peptide chain release factor C12orf65 homolog, mitochondrial K23498 COXPD7; peptide chain release factor
D 100073863 RMND1; required for meiotic nuclear division protein 1 homolog K23499 RMND1; required for meiotic nuclear division protein 1
D 100081269 GRSF1; G-rich sequence factor 1 K24984 GRSF1; G-rich sequence factor 1
D 100079996 METTL15; 12S rRNA N4-methylcytidine (m4C) methyltransferase K25876 METTL15; 12S rRNA N4-methylcytidine (m4C) methyltransferase [EC:2.1.1.-]
D 114814780 MYG1; MYG1 exonuclease K25877 MYG1; MYG1 exonuclease [EC:3.1.-.-]
D 100081022 MTRES1; mitochondrial transcription rescue factor 1 K26186 MTRES1; mitochondrial transcription rescue factor 1
B Mitochondrial DNA replication factors
C Mitochondrial DNA polymerase
D 100084569 POLG; DNA polymerase subunit gamma-1 isoform X1 K02332 POLG; DNA polymerase gamma 1 [EC:2.7.7.7]
D 100079883 POLG2; DNA polymerase subunit gamma-2, mitochondrial K02333 POLG2; DNA polymerase gamma 2
D 100084911 PRIMPOL; DNA-directed primase/polymerase protein K22761 PRIMPOL; DNA-directed primase/polymerase protein [EC:2.7.7.102 2.7.7.7]
C Mitochondrial DNA maintenance factors
D 100074231 ENDOG; endonuclease G, mitochondrial K01173 ENDOG; endonuclease G, mitochondrial
D 100085113 MGME1; mitochondrial genome maintenance exonuclease 1 K19465 MGME1; mitochondrial genome maintenance exonuclease 1 [EC:3.1.-.-]
D 100081173 twinkle protein, mitochondrial-like K17680 PEO1; twinkle protein [EC:5.6.2.3]
D 100681837 twinkle protein, mitochondrial-like isoform X1 K17680 PEO1; twinkle protein [EC:5.6.2.3]
D 114817231 twinkle protein, mitochondrial-like K17680 PEO1; twinkle protein [EC:5.6.2.3]
D 100079915 PIF1; ATP-dependent DNA helicase PIF1 K15255 PIF1; ATP-dependent DNA helicase PIF1 [EC:5.6.2.3]
C Other Mitochondrial DNA replication factors
D 100080177 SSBP1; single-stranded DNA-binding protein, mitochondrial K03111 ssb; single-strand DNA-binding protein
D 100078483 ATAD3A; ATPase family AAA domain-containing protein 3A K17681 ATAD3A_B; ATPase family AAA domain-containing protein 3A/B
D 100681553 ubiquinol-cytochrome-c reductase complex assembly factor 2 isoform X1 K17682 MNF1; mitochondrial nucleoid factor 1
D 103165630 C15H17orf80; uncharacterized protein C17orf80 homolog isoform X1 K27759 MTNAP1; mitochondrial nucleoid-associated protein 1
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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 100075633 DNAJA2; dnaJ homolog subfamily A member 2 K09503 DNAJA2; DnaJ homolog subfamily A member 2
D 100080251 RHOT1; mitochondrial Rho GTPase 1 isoform X1 K07870 RHOT1; mitochondrial Rho GTPase 1 [EC:3.6.5.-]
D 100080044 HSPA8; heat shock cognate 71 kDa protein K03283 HSPA1_6_8; heat shock 70kDa protein 1/6/8
D 100091897 heat shock 70 kDa protein 1B K03283 HSPA1_6_8; heat shock 70kDa protein 1/6/8
D 114808021 LOW QUALITY PROTEIN: heat shock 70 kDa protein 1A-like K03283 HSPA1_6_8; heat shock 70kDa protein 1/6/8
D 100080336 AIPL1; aryl-hydrocarbon-interacting protein-like 1 K17767 AIP; AH receptor-interacting protein
D 100082750 aryl-hydrocarbon-interacting protein-like 1 K17767 AIP; AH receptor-interacting protein
D 100089622 AH receptor-interacting protein K17767 AIP; AH receptor-interacting protein
D 103171084 mitochondrial fission factor homolog A K28235 FATE1; fetal and adult testis-expressed transcript protein
B Outer membrane
C Transporter outer membrane (TOM) complex
D 114807914 TOMM5; mitochondrial import receptor subunit TOM5 homolog K17773 TOM5; mitochondrial import receptor subunit TOM5
D 100083281 TOMM7; mitochondrial import receptor subunit TOM7 homolog K17771 TOM7; mitochondrial import receptor subunit TOM7
D 100081302 TOMM20; mitochondrial import receptor subunit TOM20 homolog K17770 TOM20; mitochondrial import receptor subunit TOM20
D 114816377 TOMM20L; TOMM20-like protein 1 K17770 TOM20; mitochondrial import receptor subunit TOM20
D 100090208 TOMM22; mitochondrial import receptor subunit TOM22 homolog isoform X1 K17769 TOM22; mitochondrial import receptor subunit TOM22
D 100085676 TOMM40L; mitochondrial import receptor subunit TOM40B K11518 TOM40; mitochondrial import receptor subunit TOM40
D 103165965 TOMM40; mitochondrial import receptor subunit TOM40 homolog K11518 TOM40; mitochondrial import receptor subunit TOM40
D 100089637 TOMM70; mitochondrial import receptor subunit TOM70 isoform X2 K17768 TOM70; mitochondrial import receptor subunit TOM70
C TOB/SAM complex
D 100090219 SAMM50; sorting and assembly machinery component 50 homolog isoform X1 K07277 SAM50; outer membrane protein insertion porin family
C Porin
D 100079428 VDAC1; voltage-dependent anion-selective channel protein 1 K05862 VDAC1; voltage-dependent anion channel protein 1
D 100075011 VDAC2; voltage-dependent anion-selective channel protein 2 K15040 VDAC2; voltage-dependent anion channel protein 2
D 100076584 VDAC3; voltage-dependent anion-selective channel protein 3 isoform X1 K15041 VDAC3; voltage-dependent anion channel protein 3
C ERMES complex
C Other outer membrane factor
D 100080041 MTX3; metaxin-3 isoform X1 K17776 MTX; metaxin
D 100084773 MTX2; metaxin-2 K17776 MTX; metaxin
D 100088632 MTX1; metaxin-1 isoform X1 K17776 MTX; metaxin
D 100079544 MYO19; unconventional myosin-XIX isoform X1 K25838 MYO19; myosin XIX
D 100080974 mitochondrial amidoxime-reducing component 1 K27318 MTARC; mitochondrial amidoxime-reducing component [EC:1.7.-.-]
D 114805660 mitochondrial amidoxime reducing component 2-like K27318 MTARC; mitochondrial amidoxime-reducing component [EC:1.7.-.-]
B Inter membrane space
C Transporter inner membrane (TIM) 9/10 complex
D 107547193 TIMM9; mitochondrial import inner membrane translocase subunit Tim9 K17777 TIM9; mitochondrial import inner membrane translocase subunit TIM9
D 100089677 TIMM10; mitochondrial import inner membrane translocase subunit Tim10 K17778 TIM10; mitochondrial import inner membrane translocase subunit TIM10
D 100087147 TIMM10B; mitochondrial import inner membrane translocase subunit Tim10 B K17779 TIM10B; mitochondrial import inner membrane translocase subunit TIM10B
C TIM8/13 complex
D 100082840 TIMM8A; mitochondrial import inner membrane translocase subunit Tim8 A K17780 TIM8; mitochondrial import inner membrane translocase subunit TIM8
D 100090205 TIMM8B; mitochondrial import inner membrane translocase subunit Tim8 B K17780 TIM8; mitochondrial import inner membrane translocase subunit TIM8
D 114806635 TIMM13; mitochondrial import inner membrane translocase subunit Tim13 K17781 TIM13; mitochondrial import inner membrane translocase subunit TIM13
C Mitochondrial intermembrane space assembly (MIA) complex
D 100075903 CHCHD4; mitochondrial intermembrane space import and assembly protein 40 K17782 MIA40; mitochondrial intermembrane space import and assembly protein 40
D 114806003 GFER; FAD-linked sulfhydryl oxidase ALR K17783 ERV1; mitochondrial FAD-linked sulfhydryl oxidase [EC:1.8.3.2]
B Inner mambrane
C MICOS complex
D 100085434 MICOS10; MICOS complex subunit MIC10 K17784 MICOS10; MICOS complex subunit MIC10
D 100080581 IMMT; MICOS complex subunit MIC60 isoform X1 K17785 IMMT; MICOS complex subunit MIC60
D 114806348 MICOS13; MICOS complex subunit MIC13 isoform X1 K24624 MICOS13; MICOS complex subunit MIC13
D 100075843 CHCHD3; MICOS complex subunit MIC19 isoform X3 K17563 CHCHD3; MICOS complex subunit MIC19
D 103170415 CHCHD6; MICOS complex subunit MIC25 isoform X1 K17564 CHCHD6; MICOS complex subunit MIC25
D 100082335 APOO; MICOS complex subunit MIC26 isoform X1 K24625 APOO; MICOS complex subunit MIC26
D 100087665 APOOL; MICOS complex subunit MIC27 isoform X1 K24626 APOOL; MICOS complex subunit MIC27
C MICOS complex (yeast)
D 100085434 MICOS10; MICOS complex subunit MIC10 K17784 MICOS10; MICOS complex subunit MIC10
D 100080581 IMMT; MICOS complex subunit MIC60 isoform X1 K17785 IMMT; MICOS complex subunit MIC60
C TIM22 complex
D 100078368 TIMM22; mitochondrial import inner membrane translocase subunit Tim22 K17790 TIM22; mitochondrial import inner membrane translocase subunit TIM22
C TIM23 complex
D 100091978 mitochondrial import inner membrane translocase subunit Tim23 K17794 TIM23; mitochondrial import inner membrane translocase subunit TIM23
D 100076202 TIMM17B; mitochondrial import inner membrane translocase subunit Tim17-B isoform X1 K17795 TIM17; mitochondrial import inner membrane translocase subunit TIM17
D 100076802 TIMM17A; mitochondrial import inner membrane translocase subunit Tim17-A K17795 TIM17; mitochondrial import inner membrane translocase subunit TIM17
D 100082743 TIMM21; mitochondrial import inner membrane translocase subunit Tim21 K17796 TIM21; mitochondrial import inner membrane translocase subunit TIM21
D 114812006 TIMM50; mitochondrial import inner membrane translocase subunit TIM50 isoform X1 K17496 TIM50; mitochondrial import inner membrane translocase subunit TIM50
C Mitochondrial calcium uniporter complex
D 100077394 MCU; calcium uniporter protein, mitochondrial K20858 MCU; calcium uniporter protein, mitochondrial
D 100087025 MICU1; calcium uptake protein 1, mitochondrial isoform X1 K22827 MICU1; calcium uptake protein 1, mitochondrial
D 100089599 MICU2; calcium uptake protein 2, mitochondrial K22828 MICU2; calcium uptake protein 2, mitochondrial
D 100074608 MCUB; calcium uniporter regulatory subunit MCUb, mitochondrial K22829 MCUB; calcium uniporter regulatory subunit MCUb, mitochondrial
D 100086828 SMDT1; essential MCU regulator, mitochondrial K22830 SMDT1; essential MCU regulator, mitochondrial
D 100075243 MCUR1; mitochondrial calcium uniporter regulator 1 K22137 MCUR1; mitochondrial calcium uniporter regulator 1
C Other inner membrane factors
D 100086600 LETM1; mitochondrial proton/calcium exchanger protein K17800 LETM1; LETM1 and EF-hand domain-containing protein 1, mitochondrial
D 100074482 IMMP1L; mitochondrial inner membrane protease subunit 1 isoform X1 K09647 IMP1; mitochondrial inner membrane protease subunit 1 [EC:3.4.21.-]
D 100077638 IMMP2L; mitochondrial inner membrane protease subunit 2 isoform X1 K09648 IMP2; mitochondrial inner membrane protease subunit 2 [EC:3.4.21.-]
D 100082153 TMEM126A; transmembrane protein 126A K18157 TMEM126A; transmembrane protein 126A
D 100074647 MPC1; mitochondrial pyruvate carrier 1 isoform X1 K22138 MPC1; mitochondrial pyruvate carrier 1
D 114805458 MPC1L; mitochondrial pyruvate carrier 1-like protein K22138 MPC1; mitochondrial pyruvate carrier 1
D 100083231 MPC2; mitochondrial pyruvate carrier 2 K22139 MPC2; mitochondrial pyruvate carrier 2
D 100092849 CHCHD10; coiled-coil-helix-coiled-coil-helix domain-containing protein 10, mitochondrial K22759 CHCHD10; coiled-coil-helix-coiled-coil-helix domain-containing protein 10
D 100089935 OCIAD1; OCIA domain-containing protein 1 isoform X1 K28215 OCIAD1; OCIA domain-containing protein 1
B Matrix
C MMC/PAM proteins
D 100086021 TIMM44; mitochondrial import inner membrane translocase subunit TIM44 K17804 TIM44; mitochondrial import inner membrane translocase subunit TIM44
D 100088977 HSPA9; stress-70 protein, mitochondrial K04043 dnaK; molecular chaperone DnaK
D 100076884 GRPEL2; grpE protein homolog 2, mitochondrial K03687 GRPE; molecular chaperone GrpE
D 100092501 GRPEL1; grpE protein homolog 1, mitochondrial isoform X1 K03687 GRPE; molecular chaperone GrpE
D 100074954 DNAJC19; mitochondrial import inner membrane translocase subunit TIM14 K09539 DNAJC19; DnaJ homolog subfamily C member 19
D 100084338 PAM16; mitochondrial import inner membrane translocase subunit TIM16 K17805 PAM16; mitochondrial import inner membrane translocase subunit TIM16
C Other matrix factors
D 100092395 PMPCA; mitochondrial-processing peptidase subunit alpha K01412 PMPCA; mitochondrial-processing peptidase subunit alpha [EC:3.4.24.64]
D 100076491 PMPCB; mitochondrial-processing peptidase subunit beta isoform X1 K17732 PMPCB; mitochondrial-processing peptidase subunit beta [EC:3.4.24.64]
D 100089996 mitochondrial intermediate peptidase K01410 MIPEP; mitochondrial intermediate peptidase [EC:3.4.24.59]
D 100085979 HSPD1; 60 kDa heat shock protein, mitochondrial K04077 groEL; chaperonin GroEL [EC:5.6.1.7]
D 100085990 HSPE1; 10 kDa heat shock protein, mitochondrial K04078 groES; chaperonin GroES
D 100091767 DNAJA3; dnaJ homolog subfamily A member 3, mitochondrial isoform X1 K09504 DNAJA3; DnaJ homolog subfamily A member 3
D 100074170 TAMM41; phosphatidate cytidylyltransferase, mitochondrial isoform X1 K17807 TAM41; mitochondrial translocator assembly and maintenance protein 41
D 100082558 DNLZ; DNL-type zinc finger protein K17808 ZIM17; mitochondrial protein import protein ZIM17
D 107546927 ETHE1; persulfide dioxygenase ETHE1, mitochondrial isoform X1 K17725 ETHE1; sulfur dioxygenase [EC:1.13.11.18]
D 100086752 POLDIP2; 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 100093310 CREB1; cyclic AMP-responsive element-binding protein 1 isoform X1 K05870 CREB1; cyclic AMP-responsive element-binding protein 1
D 100082870 GFM2; ribosome-releasing factor 2, mitochondrial K02355 fusA; elongation factor G
D 100086302 GFM1; elongation factor G, mitochondrial K02355 fusA; elongation factor G
D 114814764 SP1; transcription factor Sp1 K04684 SP1; transcription factor Sp1
D 100082181 YY1; transcriptional repressor protein YY1 K09201 YY; transcription factor YY
D 100073532 GABPB1; GA-binding protein subunit beta-1 isoform X1 K09454 GABPB; GA-binding protein transcription factor, beta
D 100087284 GABPB2; GA-binding protein subunit beta-2 K09454 GABPB; GA-binding protein transcription factor, beta
D 100076559 transcription factor A, mitochondrial-like K11830 TFAM; transcription factor A, mitochondrial
D 100076636 transcription factor A, mitochondrial-like K11830 TFAM; transcription factor A, mitochondrial
C Nuclear respiratory factors
D 100078954 NRF1; nuclear respiratory factor 1 isoform X1 K11831 NRF1; nuclear respiratory factor 1
D 100084872 NFE2L2; nuclear factor erythroid 2-related factor 2 isoform X1 K05638 NFE2L2; nuclear factor erythroid 2-related factor 2
D 100079807 metallothionein-like K14739 MT1_2; metallothionein 1/2
D 100073518 metallothionein-4 isoform X2 K14741 MT4; metallothionein 4
C Coactivators
D 100081496 PPARGC1A; peroxisome proliferator-activated receptor gamma coactivator 1-alpha isoform X3 K07202 PPARGC1A; alpha peroxisome proliferator-activated receptor gamma coactivator 1-alpha
D 100075366 PPARGC1B; peroxisome proliferator-activated receptor gamma coactivator 1-beta K17962 PPARGC1B; peroxisome proliferator-activated receptor gamma coactivator 1-beta
D 100080208 PPRC1; peroxisome proliferator-activated receptor gamma coactivator-related protein 1 isoform X1 K17963 PPRC1; peroxisome proliferator-activated receptor gamma coactivator-related protein 1
D 100078229 CRTC1; CREB-regulated transcription coactivator 1 isoform X1 K15309 CRTC1; CREB-regulated transcription coactivator 1
D 100076960 EP300; histone acetyltransferase p300 isoform X1 K04498 EP300; E1A/CREB-binding protein [EC:2.3.1.48]
D 100080510 CREB-binding protein isoform X1 K04498 EP300; E1A/CREB-binding protein [EC:2.3.1.48]
C Proteins involved in Fe-S cluster biogenesis
D 100085088 NFS1; cysteine desulfurase, mitochondrial K04487 iscS; cysteine desulfurase [EC:2.8.1.7]
D 100076447 ISCU; iron-sulfur cluster assembly enzyme ISCU, mitochondrial K22068 ISCU; iron-sulfur cluster assembly enzyme ISCU, mitochondrial
D 120638170 FXN; frataxin, mitochondrial K19054 FXN; frataxin [EC:1.16.3.1]
D 100077664 FDX1; adrenodoxin, mitochondrial K22070 FDX1; ferredoxin-1, mitochondrial
D 100682018 FDX2; ferredoxin-2, mitochondrial K22071 FDX2; ferredoxin-2, mitochondrial
D 114812907 adrenodoxin-like K22071 FDX2; ferredoxin-2, mitochondrial
D 100083234 FDXR; NADPH:adrenodoxin oxidoreductase, mitochondrial isoform X1 K18914 FDXR; adrenodoxin-NADP+ reductase [EC:1.18.1.6]
D 100088977 HSPA9; stress-70 protein, mitochondrial K04043 dnaK; molecular chaperone DnaK
D 114807380 SIL1; nucleotide exchange factor SIL1 K14001 SIL1; nucleotide exchange factor SIL1
D 114815245 GLRX5; glutaredoxin-related protein 5, mitochondrial K07390 grxD; monothiol glutaredoxin
D 100086719 ISCA1; iron-sulfur cluster assembly 1 homolog, mitochondrial K22063 ISCA1; iron-sulfur cluster assembly 1
D 114809241 ISCA2; LOW QUALITY PROTEIN: iron-sulfur cluster assembly 2 homolog, mitochondrial K22072 ISCA2; iron-sulfur cluster assembly 2
D 114815891 IBA57; putative transferase CAF17, mitochondrial K22073 IBA57; transferase CAF17, mitochondrial [EC:2.1.-.-]
D 100088082 NFU1; NFU1 iron-sulfur cluster scaffold homolog, mitochondrial K22074 NFU1; NFU1 iron-sulfur cluster scaffold homolog, mitochondrial
D 114808243 BOLA1; bolA-like protein 1 K22066 BOLA1; BolA-like protein 1
D 103165513 BOLA3; bolA-like protein 3 K22075 BOLA3; BolA-like protein 3
D 100080977 SFXN3; sideroflexin-3 K23500 SFXN1_3; sideroflexin-1/3
D 100081767 SFXN1; sideroflexin-1 isoform X1 K23500 SFXN1_3; sideroflexin-1/3
D 100080831 SFXN2; sideroflexin-2 isoform X1 K23501 SFXN2; sideroflexin-2
D 100084204 SFXN4; sideroflexin-4 isoform X1 K23502 SFXN4; sideroflexin-4
D 100090904 SFXN5; sideroflexin-5 isoform X1 K23503 SFXN5; sideroflexin-5
C Other regulator of mitochondrial biogenesis
D 100081561 CPT1A; carnitine O-palmitoyltransferase 1, liver isoform K08765 CPT1A; carnitine O-palmitoyltransferase 1, liver isoform [EC:2.3.1.21]
D 100073995 CPT1B; carnitine O-palmitoyltransferase 1, muscle isoform K19523 CPT1B; carnitine O-palmitoyltransferase 1, muscle isoform [EC:2.3.1.21]
D 100681838 CPT1C; carnitine O-palmitoyltransferase 1, brain isoform K19524 CPT1C; carnitine O-palmitoyltransferase 1, brain isoform [EC:2.3.1.21]
D 100085444 DHTKD1; probable 2-oxoglutarate dehydrogenase E1 component DHKTD1, mitochondrial K15791 DHKTD1; 2-oxoadipate dehydrogenase E1 component [EC:1.2.4.-]
D 100080662 FBXL4; F-box/LRR-repeat protein 4 K10270 FBXL4; F-box and leucine-rich repeat protein 4
D 100077937 HCFC1; host cell factor 1 isoform X1 K14966 HCFC1; host cell factor 1
D 100076335 HCFC2; host cell factor 2 isoform X1 K27390 HCFC2; host cell factor 2
D 103169301 HTT; LOW QUALITY PROTEIN: huntingtin K04533 HD; huntingtin
D 114809986 ESRRA; steroid hormone receptor ERR1 K08552 NR3B1; estrogen-related receptor alpha
D 100090548 ESRRB; steroid hormone receptor ERR2 isoform X1 K08553 NR3B2; estrogen-related receptor beta
D 100079572 ESRRG; estrogen-related receptor gamma isoform X1 K08554 NR3B3; estrogen-related receptor gamma
D 100080724 NRIP1; nuclear receptor-interacting protein 1 K17965 NRIP1; nuclear receptor-interacting protein 1
D 100075352 PRKAA1; 5'-AMP-activated protein kinase catalytic subunit alpha-1 isoform X1 K07198 PRKAA; 5'-AMP-activated protein kinase, catalytic alpha subunit [EC:2.7.11.31]
D 100088284 PRKAA2; 5'-AMP-activated protein kinase catalytic subunit alpha-2 isoform X1 K07198 PRKAA; 5'-AMP-activated protein kinase, catalytic alpha subunit [EC:2.7.11.31]
D 100074993 LONP1; lon protease homolog, mitochondrial K08675 PRSS15; ATP-dependent Lon protease [EC:3.4.21.53]
D 100077037 SIRT1; LOW QUALITY PROTEIN: NAD-dependent protein deacetylase sirtuin-1 K11411 SIRT1; NAD-dependent protein deacetylase sirtuin 1 [EC:2.3.1.286]
D 100074223 SERAC1; protein SERAC1 isoform X1 K23504 SERAC1; protein SERAC1
D 100088522 UXT; protein UXT isoform X1 K26537 UXT; protein UXT
D 103166298 PERM1; PGC-1 and ERR-induced regulator in muscle protein 1 K26738 PERM1; PGC-1 and ERR-induced regulator in muscle protein 1
D 100081056 ES1 protein homolog, mitochondrial-like K27835 GATD3; glutamine amidotransferase-like class 1 domain-containing protein 3, mitochondrial
D 100081540 GATD3A; glutamine amidotransferase-like class 1 domain-containing protein 3A, mitochondrial K27835 GATD3; glutamine amidotransferase-like class 1 domain-containing protein 3, mitochondrial
D 100082227 glutamine amidotransferase-like class 1 domain-containing protein 3A, mitochondrial K27835 GATD3; glutamine amidotransferase-like class 1 domain-containing protein 3, mitochondrial
D 114806079 TANGO2; transport and Golgi organization protein 2 homolog isoform X1 K28098 TANGO2; transport and Golgi organization protein 2
D 100077026 C7H2orf69; UPF0565 protein C2orf69 homolog K28271 C2ORF69; mitochondrial protein C2orf69
D 100084772 FAM214A; protein FAM214A isoform X1 K28528 ATOS; protein Atossa
D 114810099 FAM214B; protein FAM214B K28528 ATOS; protein Atossa
D 100086986 DDX47; probable ATP-dependent RNA helicase DDX47 isoform X1 K14777 DDX47; ATP-dependent RNA helicase DDX47/RRP3 [EC:5.6.2.7]
B Mitochondrial respiratory chain complex assembly factors
C Complex-I assembly factors
D 100091684 NDUFAF1; complex I intermediate-associated protein 30, mitochondrial K18159 NDUFAF1; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex assembly factor 1
D 103165346 NDUFAF2; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex assembly factor 2 K18160 NDUFAF2; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex assembly factor 2
D 100073688 NDUFAF3; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex assembly factor 3 K09008 NDUFAF3; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex assembly factor 3
D 100080229 NDUFAF4; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex assembly factor 4 K18161 NDUFAF4; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex assembly factor 4
D 100084682 NDUFAF5; arginine-hydroxylase NDUFAF5, mitochondrial isoform X1 K18162 NDUFAF5; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex assembly factor 5 [EC:2.1.1.-]
D 100073434 NDUFAF6; NADH dehydrogenase (ubiquinone) complex I, assembly factor 6 isoform X2 K18163 NDUFAF6; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex assembly factor 6
D 100083936 NDUFAF7; protein arginine methyltransferase NDUFAF7, mitochondrial isoform X1 K18164 NDUFAF7; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex assembly factor 7
D 100681792 NDUFAF8; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex assembly factor 8 isoform X1 K24726 NDUFAF8; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex assembly factor 8
D 114805880 TMEM126B; complex I assembly factor TMEM126B, mitochondrial isoform X1 K18165 TMEM126B; complex I assembly factor TMEM126B
D 100091373 ECSIT; evolutionarily conserved signaling intermediate in Toll pathway, mitochondrial K04405 ECSIT; evolutionarily conserved signaling intermediate in Toll pathway
D 100092608 ACAD9; complex I assembly factor ACAD9, mitochondrial K15980 ACAD9; acyl-CoA dehydrogenase family member 9 [EC:1.3.99.-]
D 100081894 NUBPL; iron-sulfur protein NUBPL isoform X2 K03593 mrp; ATP-binding protein involved in chromosome partitioning
D 100088363 FOXRED1; FAD-dependent oxidoreductase domain-containing protein 1 K18166 FOXRED1; FAD-dependent oxidoreductase domain-containing protein 1
D 100081267 RTN4IP1; reticulon-4-interacting protein 1, mitochondrial isoform X1 K23164 RTN4IP1; reticulon-4-interacting protein 1, mitochondrial
D 103166771 TIMMDC1; complex I assembly factor TIMMDC1, mitochondrial K23505 TIMMDC1; complex I assembly factor TIMMDC1
C Complex-II assembly factors
D 100088385 SDHAF1; succinate dehydrogenase assembly factor 1, mitochondrial K18167 SDHAF1; succinate dehydrogenase assembly factor 1
D 100073464 SDHAF2; succinate dehydrogenase assembly factor 2, mitochondrial isoform X1 K18168 SDHAF2; succinate dehydrogenase assembly factor 2
C Complex-III assembly factors
D 100088885 mitochondrial chaperone BCS1 K08900 BCS1; mitochondrial chaperone BCS1
D 100078193 TTC19; tetratricopeptide repeat protein 19, mitochondrial isoform X1 K18169 TTC19; tetratricopeptide repeat protein 19, mitochondrial
D 114808901 LYRM7; complex III assembly factor LYRM7 K18170 LYRM7; complex III assembly factor LYRM7
D 114806165 ubiquinol-cytochrome-c reductase complex assembly factor 1 K17662 CBP3; cytochrome b pre-mRNA-processing protein 3
D 100681553 ubiquinol-cytochrome-c reductase complex assembly factor 2 isoform X1 K17682 MNF1; mitochondrial nucleoid factor 1
D 114810153 ubiquinol-cytochrome-c reductase complex assembly factor 3 K23165 UQCC3; ubiquinol-cytochrome-c reductase complex assembly factor 3
D 114806102 C21H16orf91; protein CCSMST1 K27339 UQCC4; ubiquinol-cytochrome c reductase complex assembly factor 4
D 103169974 SMIM4; small integral membrane protein 4 K27340 UQCC5; ubiquinol-cytochrome c reductase complex assembly factor 5
D 100680568 C14H12orf73; protein BRAWNIN K27341 UQCC6; ubiquinol-cytochrome c reductase complex assembly factor 6
D 100681491 STMP1; short transmembrane mitochondrial protein 1 K27319 STMP1; short transmembrane mitochondrial protein 1
C Complex-IV assembly factors
D 114813805 CMC1; COX assembly mitochondrial protein homolog isoform X1 K18171 CMC1; COX assembly mitochondrial protein 1
D 114815039 CMC2; COX assembly mitochondrial protein 2 homolog K18172 CMC2; COX assembly mitochondrial protein 2
D 100076131 cytochrome c oxidase assembly factor 1 homolog K18173 COA1; cytochrome c oxidase assembly factor 1
D 114815008 cytochrome c oxidase assembly factor 3 homolog, mitochondrial K18175 CCDC56; cytochrome c oxidase assembly factor 3, animal type
D 100084288 cytochrome c oxidase assembly factor 4 homolog, mitochondrial K18177 COA4; cytochrome c oxidase assembly factor 4
D 114816935 cytochrome c oxidase assembly factor 5 K18178 COA5; cytochrome c oxidase assembly factor 5
D 100089230 cytochrome c oxidase assembly factor 6 homolog K18179 COA6; cytochrome c oxidase assembly factor 6
D 100075034 cytochrome c oxidase assembly factor 7 K18180 COA7; cytochrome c oxidase assembly factor 7
D 100078034 protoheme IX farnesyltransferase, mitochondrial K02257 COX10; heme o synthase [EC:2.5.1.141]
D 114817051 cytochrome c oxidase assembly protein COX11, mitochondrial K02258 COX11; cytochrome c oxidase assembly protein subunit 11
D 100085933 cytochrome c oxidase assembly protein COX14 K18181 COX14; cytochrome c oxidase assembly factor 14
D 100087270 cytochrome c oxidase assembly protein COX15 homolog K02259 COX15; heme a synthase [EC:1.17.99.9]
D 100090298 cytochrome c oxidase assembly protein COX16 homolog, mitochondrial K18182 COX16; cytochrome c oxidase assembly protein subunit 16
D 114817475 cytochrome c oxidase copper chaperone K02260 COX17; cytochrome c oxidase assembly protein subunit 17
D 100080784 cytochrome c oxidase assembly protein COX18, mitochondrial isoform X1 K17797 COX18; mitochondrial inner membrane protein COX18
D 100080600 cytochrome c oxidase assembly protein COX19 K18183 COX19; cytochrome c oxidase assembly protein subunit 19
D 100082973 cytochrome c oxidase assembly protein COX20, mitochondrial isoform X1 K18184 COX20; cytochrome c oxidase assembly protein subunit 20
D 103171428 MSS51; putative protein MSS51 homolog, mitochondrial isoform X1 K17656 MSS51; mitochondrial splicing suppressor protein 51
D 114806547 protein PET100 homolog, mitochondrial K18186 PET100; protein PET100, animal type
D 103171221 protein PET117 homolog, mitochondrial K18188 PET117; protein PET117
D 100086928 OXA1L; mitochondrial inner membrane protein OXA1L K03217 yidC; YidC/Oxa1 family membrane protein insertase
D 100086163 surfeit locus protein 1 isoform X1 K14998 SURF1; surfeit locus 1 family protein
D 100085276 protein SCO1 homolog, mitochondrial K07152 SCO1; protein SCO1
D 100091288 protein SCO2 homolog, mitochondrial K23755 SCO2; protein SCO2
D 100080567 translational activator of cytochrome c oxidase 1 K18189 TACO1; translational activator of cytochrome c oxidase 1
D 100083639 LRPPRC; leucine-rich PPR motif-containing protein, mitochondrial isoform X1 K17964 LRPPRC; leucine-rich PPR motif-containing protein, mitochondrial
D 100076973 cytochrome c oxidase assembly factor 8 isoform X2 K23506 APOPT1; apoptogenic protein 1
D 100681491 STMP1; short transmembrane mitochondrial protein 1 K27319 STMP1; short transmembrane mitochondrial protein 1
D 103169974 SMIM4; small integral membrane protein 4 K27340 UQCC5; ubiquinol-cytochrome c reductase complex assembly factor 5
D 100085900 TMEM223; transmembrane protein 223 K27342 TMEM223; transmembrane protein 223
D 100087657 HIGD1A; HIG1 domain family member 1A, mitochondrial K28216 HIGD1A_C; HIG1 domain family member 1A/C, mitochondrial
D 100093393 METTL7A; methyltransferase-like protein 7A isoform X3 K28216 HIGD1A_C; HIG1 domain family member 1A/C, mitochondrial
D 114807455 HIGD2A; HIG1 domain family member 2A, mitochondrial K28217 HIGD2A; HIG1 domain family member 2A, mitochondrial
C Complex-V assembly factors
D 100088238 ATPAF1; ATP synthase mitochondrial F1 complex assembly factor 1 K07555 ATPeAF1; ATP synthase mitochondrial F1 complex assembly factor 1
D 100091745 ATPAF2; ATP synthase mitochondrial F1 complex assembly factor 2 isoform X1 K07556 ATPeAF2; ATP synthase mitochondrial F1 complex assembly factor 2
D 114813171 TMEM70; transmembrane protein 70, mitochondrial isoform X1 K17966 TMEM70; transmembrane protein 70, mitochondrial
D 100076029 ATP5MJ; ATP synthase subunit ATP5MPL, mitochondrial K18193 MP68; 6.8 kDa mitochondrial proteolipid
D 100081186 ATP5MK; ATP synthase membrane subunit DAPIT, mitochondrial K18194 USMG5; up-regulated during skeletal muscle growth protein 5
B Mitochondrial dynamics
C Fission and Fusion factors
D 100073872 MFN1; mitofusin-1 K21356 MFN1; mitofusin 1 [EC:3.6.5.-]
D 100089939 MFN2; mitofusin-2 isoform X1 K06030 MFN2; mitofusin 2 [EC:3.6.5.-]
D 103170610 OPA1; dynamin-like 120 kDa protein, mitochondrial isoform X1 K17079 OPA1; optic atrophy protein 1 [EC:3.6.5.5]
D 114812126 OPA3; optic atrophy 3 protein K23166 OPA3; optic atrophy 3 protein
D 100079520 TP53-regulated inhibitor of apoptosis 1 K17968 TRIAP1; TRIAP1/MDM35 family protein
D 114809155 TRIAP1; TP53-regulated inhibitor of apoptosis 1 K17968 TRIAP1; TRIAP1/MDM35 family protein
D 100078408 PARL; presenilins-associated rhomboid-like protein, mitochondrial K09650 PARL; rhomboid-like protein [EC:3.4.21.105]
D 114813317 BAK1; bcl-2 homologous antagonist/killer isoform X1 K14021 BAK; Bcl-2 homologous antagonist/killer
D 114814649 BAX; apoptosis regulator BAX isoform X1 K02159 BAX; apoptosis regulator BAX
D 100073406 dynamin-1-like protein isoform X1 K17065 DNM1L; dynamin 1-like protein [EC:3.6.5.5]
D 100084335 DNM1L; dynamin-1-like protein isoform X1 K17065 DNM1L; dynamin 1-like protein [EC:3.6.5.5]
D 100076199 FIS1; LOW QUALITY PROTEIN: mitochondrial fission 1 protein K17969 FIS1; mitochondrial fission 1 protein
D 100075410 SUMO2; small ubiquitin-related modifier 2 K12160 SUMO; small ubiquitin-related modifier
D 100087821 SUMO3; small ubiquitin-related modifier 3 K12160 SUMO; small ubiquitin-related modifier
D 114813279 SUMO1; small ubiquitin-related modifier 1 K12160 SUMO; small ubiquitin-related modifier
D 100078296 SH3GLB1; endophilin-B1 K11248 SH3GLB1; endophilin-B1
D 100091471 MTCH1; mitochondrial carrier homolog 1 K17885 MTCH; mitochondrial carrier
D 100681186 MTCH2; mitochondrial carrier homolog 2 isoform X1 K17885 MTCH; mitochondrial carrier
D 100090080 MFF; mitochondrial fission factor isoform X1 K22076 MFF; mitochondrial fission factor
D 100083846 GDAP1L1; ganglioside-induced differentiation-associated protein 1-like 1 K22077 GDAP1; ganglioside-induced differentiation-associated protein 1
D 114813177 GDAP1; ganglioside-induced differentiation-associated protein 1 K22077 GDAP1; ganglioside-induced differentiation-associated protein 1
D 100085812 VAT1; LOW QUALITY PROTEIN: synaptic vesicle membrane protein VAT-1 homolog K23167 VAT1; synaptic vesicle membrane protein VAT-1
D 100089002 MIEF1; mitochondrial dynamics protein MID51 K23507 MIEF1_2; mitochondrial dynamics protein MID51/49
D 100093546 mitochondrial dynamics protein MID49 K23507 MIEF1_2; mitochondrial dynamics protein MID51/49
D 114809152 LOW QUALITY PROTEIN: mitochondrial dynamics protein MID49-like K23507 MIEF1_2; mitochondrial dynamics protein MID51/49
D 100080092 PLD6; mitochondrial cardiolipin hydrolase K16862 PLD6; mitochondrial cardiolipin hydrolase [EC:3.1.4.-]
D 100075000 MIGA1; mitoguardin 1 K27289 MIGA; mitoguardin
D 100088435 MIGA2; mitoguardin 2 K27289 MIGA; mitoguardin
C Actin cytoskeleton organization
D 100078865 ACTB; actin, cytoplasmic 1 K05692 ACTB_G1; actin beta/gamma 1
D 100085078 ACTG1; actin, cytoplasmic 2 K05692 ACTB_G1; actin beta/gamma 1
D 100085356 ACTL8; actin-like protein 8 K05692 ACTB_G1; actin beta/gamma 1
D 100092493 actin, cytoplasmic type 5 K05692 ACTB_G1; actin beta/gamma 1
D 100082043 NAA20; N-alpha-acetyltransferase 20 isoform X1 K17972 NAA20; N-terminal acetyltransferase B complex catalytic subunit [EC:2.3.1.254]
D 100074972 NAA25; LOW QUALITY PROTEIN: N-alpha-acetyltransferase 25, NatB auxiliary subunit K17973 NAA25; N-terminal acetyltransferase B complex non-catalytic subunit
C Mitochondrial movement
D 100082827 PHB; prohibitin K17080 PHB1; prohibitin 1
D 103164839 PHB2; prohibitin-2 K17081 PHB2; prohibitin 2
D 100082958 KTN1; kinectin isoform X1 K17975 KTN1; kinectin
C Retention of mitochondria
D 100073406 dynamin-1-like protein isoform X1 K17065 DNM1L; dynamin 1-like protein [EC:3.6.5.5]
D 100084335 DNM1L; dynamin-1-like protein isoform X1 K17065 DNM1L; dynamin 1-like protein [EC:3.6.5.5]
D 100077787 MYO5B; unconventional myosin-Vb isoform X3 K10357 MYO5; myosin V
D 100092139 MYO5C; unconventional myosin-Vc K10357 MYO5; myosin V
D 100092156 MYO5A; unconventional myosin-Va isoform X1 K10357 MYO5; myosin V
D 100079889 E3 ubiquitin-protein ligase XIAP K04725 XIAP; E3 ubiquitin-protein ligase XIAP [EC:2.3.2.27]
D 100074947 DIABLO; diablo homolog, mitochondrial K10522 DIABLO; diablo
C Morphogenesis factors
D 100087122 SNX25; sorting nexin-25 K17887 SNX25; sorting nexin-25
D 100080251 RHOT1; mitochondrial Rho GTPase 1 isoform X1 K07870 RHOT1; mitochondrial Rho GTPase 1 [EC:3.6.5.-]
D 100080581 IMMT; MICOS complex subunit MIC60 isoform X1 K17785 IMMT; MICOS complex subunit MIC60
D 114806027 MTFP1; mitochondrial fission process protein 1 K17981 MTFP1; mitochondrial fission process protein 1
D 100084231 DAP3; 28S ribosomal protein S29, mitochondrial isoform X1 K17408 DAP3; small subunit ribosomal protein S29
D 100083335 NEDD4; E3 ubiquitin-protein ligase NEDD4 isoform X1 K10591 NEDD4; E3 ubiquitin-protein ligase NEDD4 [EC:2.3.2.26]
D 100075230 OXR1; oxidation resistance protein 1 isoform X11 K25437 OXR1; oxidation resistance protein 1
D 114808052 MSTO1; protein misato homolog 1 K27320 MSTO1; protein misato
D 100089933 PRELID1; PRELI domain-containing protein 1, mitochondrial K27964 PRELID1; PRELI domain-containing protein 1, mitochondrial
D 100081154 PRELID2; PRELI domain-containing protein 2 isoform X1 K27965 PRELID2; PRELI domain-containing protein 2 mitochondrial
D 100074085 PRELID3B; PRELI domain containing protein 3B K27966 PRELID3; PRELI domain-containing protein 3, mitochondrial
D 100077351 PRELID3A; PRELI domain containing protein 3A isoform X1 K27966 PRELID3; PRELI domain-containing protein 3, mitochondrial
B Mitophagy factors
C Autophagy-related proteins
D 100081216 ATG2B; autophagy-related protein 2 homolog B isoform X1 K17906 ATG2; autophagy-related protein 2
D 100681456 ATG2A; autophagy-related protein 2 homolog A isoform X1 K17906 ATG2; autophagy-related protein 2
D 100083666 ATG3; ubiquitin-like-conjugating enzyme ATG3 K08343 ATG3; ubiquitin-like-conjugating enzyme ATG3
D 114816379 ubiquitin-like-conjugating enzyme ATG3 isoform X1 K08343 ATG3; ubiquitin-like-conjugating enzyme ATG3
D 100077214 ATG4A; cysteine protease ATG4A isoform X2 K08342 ATG4; cysteine protease ATG4 [EC:3.4.22.-]
D 100082445 ATG4B; cysteine protease ATG4B isoform X1 K08342 ATG4; cysteine protease ATG4 [EC:3.4.22.-]
D 100087854 ATG4C; cysteine protease ATG4C isoform X1 K08342 ATG4; cysteine protease ATG4 [EC:3.4.22.-]
D 100089257 ATG4D; cysteine protease ATG4D K08342 ATG4; cysteine protease ATG4 [EC:3.4.22.-]
D 100081430 ATG5; autophagy protein 5 K08339 ATG5; autophagy-related protein 5
D 100083983 BECN1; beclin-1 isoform X1 K08334 BECN; beclin
D 100077565 ATG7; ubiquitin-like modifier-activating enzyme ATG7 isoform X1 K08337 ATG7; ubiquitin-like modifier-activating enzyme ATG7
D 100077391 gamma-aminobutyric acid receptor-associated protein-like 2 K08341 GABARAP; GABA(A) receptor-associated protein
D 100083105 gamma-aminobutyric acid receptor-associated protein K08341 GABARAP; GABA(A) receptor-associated protein
D 100085684 GABARAPL1; gamma-aminobutyric acid receptor-associated protein-like 1 K08341 GABARAP; GABA(A) receptor-associated protein
D 114815189 gamma-aminobutyric acid receptor-associated protein-like 2 K08341 GABARAP; GABA(A) receptor-associated protein
D 100085629 ATG9B; autophagy-related protein 9B K17907 ATG9; autophagy-related protein 9
D 100081058 ATG10; ubiquitin-like-conjugating enzyme ATG10 K17888 ATG10L; ubiquitin-like-conjugating enzyme ATG10
D 100681077 ATG14; beclin 1-associated autophagy-related key regulator K17889 ATG14L; beclin 1-associated autophagy-related key regulator
D 100092288 ATG16L1; autophagy-related protein 16-1 isoform X1 K17890 ATG16L1; autophagy-related protein 16-1
D 100081762 ATG16L2; protein Atg16l2 isoform X1 K20868 ATG16L2; autophagy-related protein 16-2
D 100078638 WIPI1; WD repeat domain phosphoinositide-interacting protein 1 isoform X1 K17908 WIPI1_2; autophagy-related protein 18
D 100082003 WIPI2; WD repeat domain phosphoinositide-interacting protein 2 K17908 WIPI1_2; autophagy-related protein 18
C Parkin-dependent mechanism factors
D 114809162 PRKN; E3 ubiquitin-protein ligase parkin K04556 PARK2; parkin [EC:2.3.2.31]
D 100086922 RNF41; E3 ubiquitin-protein ligase NRDP1 K11981 RNF41; E3 ubiquitin-protein ligase NRDP1 [EC:2.3.2.27]
D 100078362 CLEC16A; protein CLEC16A isoform X1 K19513 CLEC16A; protein CLEC16A
D 100089816 PINK1; serine/threonine-protein kinase PINK1, mitochondrial K05688 PINK1; PTEN induced putative kinase 1 [EC:2.7.11.1]
D 100078408 PARL; presenilins-associated rhomboid-like protein, mitochondrial K09650 PARL; rhomboid-like protein [EC:3.4.21.105]
D 100074478 SQSTM1; sequestosome-1 isoform X2 K14381 SQSTM1; sequestosome 1
D 100077721 TAX1BP1; tax1-binding protein 1 isoform X1 K21347 TAX1BP1; Tax1-binding protein 1
D 100682151 CALCOCO2; calcium-binding and coiled-coil domain-containing protein 2 K21348 CALCOCO2; calcium-binding and coiled-coil domain-containing protein 2
D 100093574 AMBRA1; LOW QUALITY PROTEIN: activating molecule in BECN1-regulated autophagy protein 1 K17985 AMBRA1; activating molecule in BECN1-regulated autophagy protein 1
C Parkin-independent mechanism factors
D 100087432 ATG13; autophagy-related protein 13 isoform X1 K08331 ATG13; autophagy-related protein 13
D 100093196 ULK1; serine/threonine-protein kinase ULK1 isoform X1 K21357 ULK1; serine/threonine-protein kinase ULK1 [EC:2.7.11.1]
D 100077170 ULK2; serine/threonine-protein kinase ULK2 K08269 ULK2; serine/threonine-protein kinase ULK2 [EC:2.7.11.1]
D 100079581 RB1CC1; RB1-inducible coiled-coil protein 1 isoform X1 K17589 RB1CC1; RB1-inducible coiled-coil protein 1
D 100082891 HSP90AA1; heat shock protein HSP 90-alpha K04079 HSP90A; molecular chaperone HtpG
D 100089224 HSP90AB1; heat shock protein HSP 90-beta K04079 HSP90A; molecular chaperone HtpG
D 100081344 CDC37; hsp90 co-chaperone Cdc37 K09554 CDC37; cell division cycle protein 37
D 100079802 SMURF2; E3 ubiquitin-protein ligase SMURF2 K04678 SMURF; E3 ubiquitin ligase SMURF1/2 [EC:2.3.2.26]
D 100081555 SMURF1; E3 ubiquitin-protein ligase SMURF1 isoform X1 K04678 SMURF; E3 ubiquitin ligase SMURF1/2 [EC:2.3.2.26]
D 100077141 BNIP3L; LOW QUALITY PROTEIN: BCL2/adenovirus E1B 19 kDa protein-interacting protein 3-like K15465 BNIP3L; BCL2/adenovirus E1B 19 kDa protein-interacting protein 3-like
D 100081877 FUNDC1; FUN14 domain-containing protein 1 K17986 FUNDC1; FUN14 domain-containing protein 1
C Other mitophagy factors
D 100075352 PRKAA1; 5'-AMP-activated protein kinase catalytic subunit alpha-1 isoform X1 K07198 PRKAA; 5'-AMP-activated protein kinase, catalytic alpha subunit [EC:2.7.11.31]
D 100088284 PRKAA2; 5'-AMP-activated protein kinase catalytic subunit alpha-2 isoform X1 K07198 PRKAA; 5'-AMP-activated protein kinase, catalytic alpha subunit [EC:2.7.11.31]
D 100082738 MAP1LC3B; microtubule-associated proteins 1A/1B light chain 3B K10435 MAP1LC; microtubule-associated protein 1 light chain
D 114816899 MAP1LC3A; microtubule-associated proteins 1A/1B light chain 3A K10435 MAP1LC; microtubule-associated protein 1 light chain
D 100088445 NBR1; next to BRCA1 gene 1 protein isoform X1 K17987 NBR1; next to BRCA1 gene 1 protein
D 100081554 TECPR1; tectonin beta-propeller repeat-containing protein 1 isoform X1 K17988 TECPR1; tectonin beta-propeller repeat-containing protein 1
D 100081199 zinc finger FYVE domain-containing protein 1-like K17603 ZFYVE1; zinc finger FYVE domain-containing protein 1
D 100090119 ZFYVE1; zinc finger FYVE domain-containing protein 1 isoform X1 K17603 ZFYVE1; zinc finger FYVE domain-containing protein 1
D 100680675 COASY; LOW QUALITY PROTEIN: bifunctional coenzyme A synthase K02318 COASY; phosphopantetheine adenylyltransferase / dephospho-CoA kinase [EC:2.7.7.3 2.7.1.24]
D 100090638 PLA2G6; 85/88 kDa calcium-independent phospholipase A2 isoform X2 K16343 PLA2G6; calcium-independent phospholipase A2 [EC:3.1.1.4]
D 100681902 C11H19orf12; protein C19orf12 homolog isoform X1 K23168 MPAN; protein MPAN
D 100084124 FUNDC2; FUN14 domain-containing protein 2 K24308 FUNDC2; FUN14 domain-containing protein 2
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#[ BRITE | KEGG2 | KEGG ]
#Last updated: September 5, 2025