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Mitochondrial Biogenesis - Oryctolagus cuniculus (rabbit)
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AMitochondrial DNA transcription, translation, and replication factors
B Mitochondrial DNA-encoded proteins
C Mitochondrial respiratory chain complex I
D 808226 ND1; NADH dehydrogenase subunit 1 K03878 ND1; NADH-ubiquinone oxidoreductase chain 1 [EC:7.1.1.2]
D 808230 ND2; NADH dehydrogenase subunit 2 K03879 ND2; NADH-ubiquinone oxidoreductase chain 2 [EC:7.1.1.2]
D 808221 ND3; NADH dehydrogenase subunit 3 K03880 ND3; NADH-ubiquinone oxidoreductase chain 3 [EC:7.1.1.2]
D 808225 ND4; NADH dehydrogenase subunit 4 K03881 ND4; NADH-ubiquinone oxidoreductase chain 4 [EC:7.1.1.2]
D 808222 ND4L; NADH dehydrogenase subunit 4L K03882 ND4L; NADH-ubiquinone oxidoreductase chain 4L [EC:7.1.1.2]
D 808224 ND5; NADH dehydrogenase subunit 5 K03883 ND5; NADH-ubiquinone oxidoreductase chain 5 [EC:7.1.1.2]
D 808223 ND6; NADH dehydrogenase subunit 6 K03884 ND6; NADH-ubiquinone oxidoreductase chain 6 [EC:7.1.1.2]
C Mitochondrial respiratory chain complex III
D 808227 CYTB; cytochrome b K00412 CYTB; ubiquinol-cytochrome c reductase cytochrome b subunit
C Cytochrome c oxidase
D 808231 COX1; cytochrome c oxidase subunit I K02256 COX1; cytochrome c oxidase subunit 1 [EC:7.1.1.9]
D 808233 COX2; cytochrome c oxidase subunit II K02261 COX2; cytochrome c oxidase subunit 2
D 808229 COX3; cytochrome c oxidase subunit III K02262 COX3; cytochrome c oxidase subunit 3
C F-type ATPase
D 808228 ATP8; ATP synthase F0 subunit 8 K02125 ATPeF08; F-type H+-transporting ATPase subunit 8
D 808232 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 100353467 LOW QUALITY PROTEIN: DNA-directed RNA polymerase, mitochondrial K10908 POLRMT; DNA-directed RNA polymerase, mitochondrial [EC:2.7.7.6]
C Mitochondrial transcription factors
D 100358902 transcription factor A, mitochondrial K11830 TFAM; transcription factor A, mitochondrial
D 100352013 dimethyladenosine transferase 1, mitochondrial isoform X2 K15266 TFB1M; dimethyladenosine transferase 1, mitochondrial [EC:2.1.1.-]
D 100346999 dimethyladenosine transferase 2, mitochondrial isoform X1 K17653 TFB2M; dimethyladenosine transferase 2, mitochondrial [EC:2.1.1.-]
D 100357810 dimethyladenosine transferase 2, mitochondrial-like K17653 TFB2M; dimethyladenosine transferase 2, mitochondrial [EC:2.1.1.-]
C Mitochondrial translation factors
D 100359110 translation initiation factor IF-2, mitochondrial K02519 infB; translation initiation factor IF-2
D 103347364 translation initiation factor IF-2, mitochondrial isoform X1 K02519 infB; translation initiation factor IF-2
D 100339602 translation initiation factor IF-3, mitochondrial isoform X1 K02520 infC; translation initiation factor IF-3
D 100347350 elongation factor Ts, mitochondrial K02357 tsf; elongation factor Ts
D 127486365 elongation factor Ts, mitochondrial K02357 tsf; elongation factor Ts
D 100344982 elongation factor Tu, mitochondrial K02358 tuf; elongation factor Tu
D 103347974 elongation factor Tu, mitochondrial-like K02358 tuf; elongation factor Tu
D 100344691 ribosome-releasing factor 2, mitochondrial isoform X1 K02355 fusA; elongation factor G
D 100345180 elongation factor G, mitochondrial K02355 fusA; elongation factor G
D 100338222 transcription termination factor 3, mitochondrial K15031 MTERF; mitochondrial transcription termination factor family protein
D 100345400 transcription termination factor 4, mitochondrial isoform X1 K15031 MTERF; mitochondrial transcription termination factor family protein
D 100347306 transcription termination factor 2, mitochondrial K15031 MTERF; mitochondrial transcription termination factor family protein
D 100355865 transcription termination factor 1, mitochondrial K15031 MTERF; mitochondrial transcription termination factor family protein
D 100338633 translation factor GUF1, mitochondrial isoform X1 K21594 GUF1; translation factor GUF1, mitochondrial [EC:3.6.5.-]
C RNase MRP
D 100339220 ribonucleases P/MRP protein subunit POP1 K01164 POP1; ribonuclease P/MRP protein subunit POP1 [EC:3.1.26.5]
D 100351294 ribonuclease P protein subunit p29 isoform X1 K03538 POP4; ribonuclease P protein subunit POP4 [EC:3.1.26.5]
D 100351855 ribonuclease P/MRP protein subunit POP5 K03537 POP5; ribonuclease P/MRP protein subunit POP5 [EC:3.1.26.5]
D 127488125 ribonuclease P/MRP protein subunit POP5 K03537 POP5; ribonuclease P/MRP protein subunit POP5 [EC:3.1.26.5]
D 100340340 ribonuclease P protein subunit p30 K03539 RPP1; ribonuclease P/MRP protein subunit RPP1 [EC:3.1.26.5]
D 100341086 ribonuclease P protein subunit p20 K14527 RPP20; ribonuclease P/MRP protein subunit RPP20 [EC:3.1.26.5]
D 100342169 ribonuclease P protein subunit p25-like protein K14525 RPP25; ribonucleases P/MRP protein subunit RPP25 [EC:3.1.26.5]
D 100343857 ribonuclease P protein subunit p25 K14525 RPP25; ribonucleases P/MRP protein subunit RPP25 [EC:3.1.26.5]
D 100343235 ribonuclease P protein subunit p38 K14523 RPP38; ribonucleases P/MRP protein subunit RPP38 [EC:3.1.26.5]
D 100351937 ribonuclease P protein subunit p38 K14523 RPP38; ribonucleases P/MRP protein subunit RPP38 [EC:3.1.26.5]
D 100351447 ribonuclease P protein subunit p40 K14530 RPP40; ribonucleases P/MRP protein subunit RPP40 [EC:3.1.26.5]
C Mitochondrial ribonuclease P
D 100351020 tRNA methyltransferase 10 homolog C K17654 TRMT10C; mitochondrial ribonuclease P protein 1 [EC:2.1.1.-]
D 100342235 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 100339508 PRORP; mitochondrial ribonuclease P catalytic subunit isoform X1 K17655 MRPP3; mitochondrial ribonuclease P protein 3
C Other mitochondrial DNA transcription and translation factors
D 100353370 transcription elongation factor, mitochondrial K17658 TEFM; transcription elongation factor, mitochondrial
D 100352550 pentatricopeptide repeat-containing protein 1, mitochondrial K17710 PTCD1; pentatricopeptide repeat domain-containing protein 1
D 100342155 pentatricopeptide repeat-containing protein 2, mitochondrial isoform X1 K17711 PTCD2; pentatricopeptide repeat domain-containing protein 2
D 100337958 pentatricopeptide repeat domain-containing protein 3, mitochondrial isoform X1 K17659 PTCD3; pentatricopeptide repeat domain-containing protein 3
D 100345961 LOW QUALITY PROTEIN: pentatricopeptide repeat domain-containing protein 3, mitochondrial-like K17659 PTCD3; pentatricopeptide repeat domain-containing protein 3
D 100354031 ATP-dependent RNA helicase DHX30 isoform X1 K13185 DHX30; ATP-dependent RNA helicase DHX30 [EC:5.6.2.6]
D 100355688 ubiquinol-cytochrome-c reductase complex assembly factor 1 isoform X1 K17662 CBP3; cytochrome b pre-mRNA-processing protein 3
D 100345087 AFG1L; AFG1-like ATPase isoform X1 K18798 AFG1; peroxisome-assembly ATPase [EC:3.6.4.7]
D 100347852 mitochondrial inner membrane protease ATP23 homolog isoform X1 K18156 ATP23; mitochondrial inner membrane protease ATP23 [EC:3.4.24.-]
D 100345360 ATP-dependent RNA helicase SUPV3L1, mitochondrial isoform X1 K17675 SUPV3L1; ATP-dependent RNA helicase SUPV3L1/SUV3 [EC:5.6.2.6]
D 100347971 endoribonuclease LACTB2 K16639 LACTB2; endoribonuclease LACTB2 [EC:3.1.27.-]
D 100009414 SLC25A4; ADP/ATP translocase 1 K05863 SLC25A4S; solute carrier family 25 (mitochondrial adenine nucleotide translocator), member 4/5/6/31
D 100337764 ADP/ATP translocase 2 K05863 SLC25A4S; solute carrier family 25 (mitochondrial adenine nucleotide translocator), member 4/5/6/31
D 100342972 ADP/ATP translocase 2-like K05863 SLC25A4S; solute carrier family 25 (mitochondrial adenine nucleotide translocator), member 4/5/6/31
D 100346477 ADP/ATP translocase 4 isoform X1 K05863 SLC25A4S; solute carrier family 25 (mitochondrial adenine nucleotide translocator), member 4/5/6/31
D 100348057 ADP/ATP translocase 2-like K05863 SLC25A4S; solute carrier family 25 (mitochondrial adenine nucleotide translocator), member 4/5/6/31
D 100349768 ADP/ATP translocase 1 K05863 SLC25A4S; solute carrier family 25 (mitochondrial adenine nucleotide translocator), member 4/5/6/31
D 100354802 ADP/ATP translocase 2-like K05863 SLC25A4S; solute carrier family 25 (mitochondrial adenine nucleotide translocator), member 4/5/6/31
D 100356326 ADP/ATP translocase 2 K05863 SLC25A4S; solute carrier family 25 (mitochondrial adenine nucleotide translocator), member 4/5/6/31
D 100342228 mitoferrin-2 K15113 SLC25A28_37; solute carrier family 25 (mitochondrial iron transporter), member 28/37
D 100345144 mitoferrin-1 isoform X1 K15113 SLC25A28_37; solute carrier family 25 (mitochondrial iron transporter), member 28/37
D 100350824 mitochondrial assembly of ribosomal large subunit protein 1 K25803 MALSU1; mitochondrial assembly of ribosomal large subunit protein 1
D 100348774 GTPase Era, mitochondrial isoform X1 K03595 era; GTPase
D 100338314 tyrosine--tRNA ligase, mitochondrial K01866 YARS; tyrosyl-tRNA synthetase [EC:6.1.1.1]
D 100353312 YARS1; tyrosine--tRNA ligase, cytoplasmic K01866 YARS; tyrosyl-tRNA synthetase [EC:6.1.1.1]
D 100358356 leucine--tRNA ligase, mitochondrial isoform X2 K01869 LARS; leucyl-tRNA synthetase [EC:6.1.1.4]
D 100359202 LARS1; leucine--tRNA ligase, cytoplasmic K01869 LARS; leucyl-tRNA synthetase [EC:6.1.1.4]
D 100344833 aspartate--tRNA ligase, mitochondrial isoform X1 K01876 DARS2; aspartyl-tRNA synthetase [EC:6.1.1.12]
D 100338153 GARS1; glycine--tRNA ligase K01880 GARS; glycyl-tRNA synthetase [EC:6.1.1.14]
D 100338051 RARS1; arginine--tRNA ligase, cytoplasmic K01887 RARS; arginyl-tRNA synthetase [EC:6.1.1.19]
D 100348236 probable arginine--tRNA ligase, mitochondrial isoform X1 K01887 RARS; arginyl-tRNA synthetase [EC:6.1.1.19]
D 100339740 glutamyl-tRNA(Gln) amidotransferase subunit A, mitochondrial K02433 gatA; aspartyl-tRNA(Asn)/glutamyl-tRNA(Gln) amidotransferase subunit A [EC:6.3.5.6 6.3.5.7]
D 100356898 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 100346657 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 100355312 poly(A) RNA polymerase, mitochondrial isoform X1 K18060 MTPAP; poly(A) RNA polymerase, mitochondrial [EC:2.7.7.19]
D 100353603 2',5'-phosphodiesterase 12 K19612 PDE12; 2',5'-phosphodiesterase [EC:3.1.13.4 3.1.4.-]
D 100340781 mitochondrial ribosome-associated GTPase 1 isoform X3 K19828 MTG1; mitochondrial GTPase 1
D 100341545 nitric oxide-associated protein 1 isoform X1 K19832 NOA1; nitric oxide-associated protein 1
D 100343486 tRNA (cytosine(34)-C(5))-methyltransferase, mitochondrial isoform X3 K21969 NSUN3; tRNA (cytosine34-C5)-methyltransferase [EC:2.1.1.-]
D 100343475 5-methylcytosine rRNA methyltransferase NSUN4 isoform X2 K21970 NSUN4; 5-methylcytosine rRNA methyltransferase NSUN4 [EC:2.1.1.-]
D 100351049 mitochondrial mRNA pseudouridine synthase RPUSD3 isoform X2 K22537 RPUSD3; mitochondrial RNA pseudouridine synthase RPUSD3 [EC:5.4.99.-]
D 100339442 pseudouridylate synthase RPUSD4, mitochondrial isoform X2 K22538 RPUSD4; mitochondrial RNA pseudouridine synthase RPUSD4 [EC:5.4.99.-]
D 100339190 RCC1-like G exchanging factor-like protein isoform X1 K23495 RCC1L; RCC1-like G exchanging factor-like protein
D 127489593 RCC1-like G exchanging factor-like protein isoform X1 K23495 RCC1L; RCC1-like G exchanging factor-like protein
D 100340285 neugrin K23496 NGRN; neugrin
D 100355666 pseudouridylate synthase TRUB2, mitochondrial K23497 TRUB2; mitochondrial mRNA pseudouridine synthase TRUB2 [EC:5.4.99.-]
D 100346792 FAST kinase domain-containing protein 1, mitochondrial isoform X1 K27754 FASTKD1; FAST kinase domain-containing protein 1
D 100351224 FAST kinase domain-containing protein 2, mitochondrial-like K18190 FASTKD2; FAST kinase domain-containing protein 2
D 100355694 LOW QUALITY PROTEIN: FAST kinase domain-containing protein 2, mitochondrial K18190 FASTKD2; FAST kinase domain-containing protein 2
D 100350367 FAST kinase domain-containing protein 3, mitochondrial K27755 FASTKD3; FAST kinase domain-containing protein 3
D 100338656 FAST kinase domain-containing protein 4 K27756 TBRG4; FAST kinase domain-containing protein 4
D 100344307 FAST kinase domain-containing protein 5, mitochondrial K27757 FASTKD5; FAST kinase domain-containing protein 5
D 100347368 MTRFR; mitochondrial translation release factor in rescue K23498 COXPD7; peptide chain release factor
D 100348491 required for meiotic nuclear division protein 1 homolog isoform X1 K23499 RMND1; required for meiotic nuclear division protein 1
D 100349091 G-rich sequence factor 1 isoform X1 K24984 GRSF1; G-rich sequence factor 1
D 100357205 12S rRNA N4-methylcytidine (m4C) methyltransferase isoform X1 K25876 METTL15; 12S rRNA N4-methylcytidine (m4C) methyltransferase [EC:2.1.1.-]
D 100351065 MYG1; MYG1 exonuclease K25877 MYG1; MYG1 exonuclease [EC:3.1.-.-]
D 100340244 MTRES1; mitochondrial transcription rescue factor 1 isoform X2 K26186 MTRES1; mitochondrial transcription rescue factor 1
B Mitochondrial DNA replication factors
C Mitochondrial DNA polymerase
D 100347697 DNA polymerase subunit gamma-1 isoform X1 K02332 POLG; DNA polymerase gamma 1 [EC:2.7.7.7]
D 100351035 DNA polymerase subunit gamma-2, mitochondrial isoform X1 K02333 POLG2; DNA polymerase gamma 2
D 100342519 DNA-directed primase/polymerase protein isoform X1 K22761 PRIMPOL; DNA-directed primase/polymerase protein [EC:2.7.7.102 2.7.7.7]
D 103347387 DNA-directed primase/polymerase protein isoform X1 K22761 PRIMPOL; DNA-directed primase/polymerase protein [EC:2.7.7.102 2.7.7.7]
D 103347617 DNA-directed primase/polymerase protein-like isoform X1 K22761 PRIMPOL; DNA-directed primase/polymerase protein [EC:2.7.7.102 2.7.7.7]
D 127487426 DNA-directed primase/polymerase protein isoform X1 K22761 PRIMPOL; DNA-directed primase/polymerase protein [EC:2.7.7.102 2.7.7.7]
C Mitochondrial DNA maintenance factors
D 100350715 endonuclease G, mitochondrial K01173 ENDOG; endonuclease G, mitochondrial
D 100357051 mitochondrial genome maintenance exonuclease 1 isoform X1 K19465 MGME1; mitochondrial genome maintenance exonuclease 1 [EC:3.1.-.-]
D 103347242 nuclease EXOG, mitochondrial isoform X1 K15050 EXOG; nuclease EXOG, mitochondrial
D 100349273 TWNK; twinkle mtDNA helicase K17680 PEO1; twinkle protein [EC:5.6.2.3]
D 100351596 ATP-dependent DNA helicase PIF1 K15255 PIF1; ATP-dependent DNA helicase PIF1 [EC:5.6.2.3]
C Other Mitochondrial DNA replication factors
D 100008866 SSBP1; single-stranded DNA-binding protein, mitochondrial precursor K03111 ssb; single-strand DNA-binding protein
D 100342744 single-stranded DNA-binding protein, mitochondrial-like K03111 ssb; single-strand DNA-binding protein
D 100357130 single-stranded DNA-binding protein, mitochondrial-like K03111 ssb; single-strand DNA-binding protein
D 127490310 single-stranded DNA-binding protein, mitochondrial-like K03111 ssb; single-strand DNA-binding protein
D 103347023 ATPase family AAA domain-containing protein 3 isoform X1 K17681 ATAD3A_B; ATPase family AAA domain-containing protein 3A/B
D 100355204 ubiquinol-cytochrome-c reductase complex assembly factor 2 K17682 MNF1; mitochondrial nucleoid factor 1
D 100342072 C19H17orf80; uncharacterized protein C17orf80 homolog isoform X2 K27759 MTNAP1; mitochondrial nucleoid-associated protein 1
D 100342498 GTPase IMAP family member 1 isoform X1 K27758 GIMAP1_3_5; GTPase IMAP family member 1/3/5
D 100343263 LOW QUALITY PROTEIN: uncharacterized protein LOC100343263 K27758 GIMAP1_3_5; GTPase IMAP family member 1/3/5
D 100347861 GTPase IMAP family member 5 K27758 GIMAP1_3_5; GTPase IMAP family member 1/3/5
D 100348108 GTPase IMAP family member 5 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 100350952 dnaJ homolog subfamily A member 2 K09503 DNAJA2; DnaJ homolog subfamily A member 2
D 100358561 mitochondrial import receptor subunit TOM34 isoform X1 K17766 TOM34; mitochondrial import receptor subunit TOM34
D 100351349 mitochondrial Rho GTPase 1 isoform X3 K07870 RHOT1; mitochondrial Rho GTPase 1 [EC:3.6.5.-]
D 100338160 LOW QUALITY PROTEIN: heat shock 70 kDa protein 6 K03283 HSPA1_6_8; heat shock 70kDa protein 1/6/8
D 100348873 heat shock cognate 71 kDa protein K03283 HSPA1_6_8; heat shock 70kDa protein 1/6/8
D 100353534 heat shock 70 kDa protein 1-like K03283 HSPA1_6_8; heat shock 70kDa protein 1/6/8
D 100354037 heat shock 70 kDa protein 1B K03283 HSPA1_6_8; heat shock 70kDa protein 1/6/8
D 100354435 heat shock 70 kDa protein 1B K03283 HSPA1_6_8; heat shock 70kDa protein 1/6/8
D 100356572 heat shock cognate 71 kDa protein K03283 HSPA1_6_8; heat shock 70kDa protein 1/6/8
D 127488889 heat shock 70 kDa protein 1B K03283 HSPA1_6_8; heat shock 70kDa protein 1/6/8
D 127488890 heat shock 70 kDa protein 1B-like K03283 HSPA1_6_8; heat shock 70kDa protein 1/6/8
D 127493068 heat shock 70 kDa protein 1B-like K03283 HSPA1_6_8; heat shock 70kDa protein 1/6/8
D 100347082 aryl-hydrocarbon-interacting protein-like 1 K17767 AIP; AH receptor-interacting protein
B Outer membrane
C Transporter outer membrane (TOM) complex
D 100349412 mitochondrial import receptor subunit TOM40B K11518 TOM40; mitochondrial import receptor subunit TOM40
D 127485451 mitochondrial import receptor subunit TOM40 homolog K11518 TOM40; mitochondrial import receptor subunit TOM40
D 100352274 mitochondrial import receptor subunit TOM70 K17768 TOM70; mitochondrial import receptor subunit TOM70
D 103348393 mitochondrial import receptor subunit TOM22 homolog K17769 TOM22; mitochondrial import receptor subunit TOM22
D 100341031 mitochondrial import receptor subunit TOM20 homolog isoform X4 K17770 TOM20; mitochondrial import receptor subunit TOM20
D 100342885 mitochondrial import receptor subunit TOM20 homolog K17770 TOM20; mitochondrial import receptor subunit TOM20
D 100352857 mitochondrial import receptor subunit TOM20 homolog K17770 TOM20; mitochondrial import receptor subunit TOM20
D 100352955 TOMM20-like protein 1 isoform X2 K17770 TOM20; mitochondrial import receptor subunit TOM20
D 100337985 mitochondrial import receptor subunit TOM7 homolog isoform X3 K17771 TOM7; mitochondrial import receptor subunit TOM7
D 108175599 mitochondrial import receptor subunit TOM7 homolog K17771 TOM7; mitochondrial import receptor subunit TOM7
D 108178455 mitochondrial import receptor subunit TOM7 homolog K17771 TOM7; mitochondrial import receptor subunit TOM7
D 100328716 TOMM6; mitochondrial import receptor subunit TOM6 homolog K17772 TOM6; mitochondrial import receptor subunit TOM6
D 100343944 mitochondrial import receptor subunit TOM5 homolog K17773 TOM5; mitochondrial import receptor subunit TOM5
C TOB/SAM complex
D 100345138 sorting and assembly machinery component 50 homolog K07277 SAM50; outer membrane protein insertion porin family
C Porin
D 100008751 VDAC1; voltage-dependent anion-selective channel protein 1 K05862 VDAC1; voltage-dependent anion channel protein 1
D 100349506 LOW QUALITY PROTEIN: voltage-dependent anion-selective channel protein 1-like K05862 VDAC1; voltage-dependent anion channel protein 1
D 100009473 VDAC2; voltage-dependent anion-selective channel protein 2 K15040 VDAC2; voltage-dependent anion channel protein 2
D 100008752 VDAC3; voltage-dependent anion-selective channel protein 3 K15041 VDAC3; voltage-dependent anion channel protein 3
C ERMES complex
C Other outer membrane factor
D 100337978 metaxin-2 isoform X1 K17776 MTX; metaxin
D 100356630 metaxin-3 isoform X1 K17776 MTX; metaxin
D 100357146 metaxin-1 K17776 MTX; metaxin
D 100357003 unconventional myosin-XIX isoform X1 K25838 MYO19; myosin XIX
D 100351951 armadillo repeat-containing X-linked protein 1 K26187 ARMCX1; armadillo repeat-containing X-linked protein 1
D 100343345 armadillo repeat-containing X-linked protein 2 K26188 ARMCX2; armadillo repeat-containing X-linked protein 2
D 100349702 armadillo repeat-containing X-linked protein 3 K26189 ARMCX3; armadillo repeat-containing X-linked protein 3
D 100350443 protein ARMCX6 K26190 ARMCX6; armadillo repeat-containing X-linked protein 6
D 100352526 MTARC1; mitochondrial amidoxime-reducing component 1 isoform X2 K27318 MTARC; mitochondrial amidoxime-reducing component [EC:1.7.-.-]
D 100352787 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 100338928 mitochondrial import inner membrane translocase subunit Tim9 K17777 TIM9; mitochondrial import inner membrane translocase subunit TIM9
D 100349306 mitochondrial import inner membrane translocase subunit Tim10 K17778 TIM10; mitochondrial import inner membrane translocase subunit TIM10
D 100355100 mitochondrial import inner membrane translocase subunit Tim10 B K17779 TIM10B; mitochondrial import inner membrane translocase subunit TIM10B
C TIM8/13 complex
D 100346423 mitochondrial import inner membrane translocase subunit Tim8 B K17780 TIM8; mitochondrial import inner membrane translocase subunit TIM8
D 100348662 mitochondrial import inner membrane translocase subunit Tim8 A-like K17780 TIM8; mitochondrial import inner membrane translocase subunit TIM8
D 100355732 mitochondrial import inner membrane translocase subunit Tim8 A K17780 TIM8; mitochondrial import inner membrane translocase subunit TIM8
C Mitochondrial intermembrane space assembly (MIA) complex
D 100345760 mitochondrial intermembrane space import and assembly protein 40 isoform X2 K17782 MIA40; mitochondrial intermembrane space import and assembly protein 40
D 127486397 FAD-linked sulfhydryl oxidase ALR isoform X1 K17783 ERV1; mitochondrial FAD-linked sulfhydryl oxidase [EC:1.8.3.2]
B Inner mambrane
C MICOS complex
D 100352683 MICOS10; MICOS complex subunit MIC10 K17784 MICOS10; MICOS complex subunit MIC10
D 100337704 MICOS complex subunit MIC60 isoform X1 K17785 IMMT; MICOS complex subunit MIC60
D 100347311 MICOS complex subunit MIC19 isoform X1 K17563 CHCHD3; MICOS complex subunit MIC19
D 100358523 MICOS complex subunit MIC25 isoform X3 K17564 CHCHD6; MICOS complex subunit MIC25
D 100343342 MICOS complex subunit MIC26 isoform X3 K24625 APOO; MICOS complex subunit MIC26
D 100359034 MICOS complex subunit MIC26-like K24625 APOO; MICOS complex subunit MIC26
D 103347047 MICOS complex subunit MIC26-like K24625 APOO; MICOS complex subunit MIC26
D 100350289 MICOS complex subunit MIC27 K24626 APOOL; MICOS complex subunit MIC27
C MICOS complex (yeast)
D 100352683 MICOS10; MICOS complex subunit MIC10 K17784 MICOS10; MICOS complex subunit MIC10
D 100337704 MICOS complex subunit MIC60 isoform X1 K17785 IMMT; MICOS complex subunit MIC60
C TIM22 complex
D 100338681 mitochondrial import inner membrane translocase subunit Tim22 K17790 TIM22; mitochondrial import inner membrane translocase subunit TIM22
C TIM23 complex
D 100341303 mitochondrial import inner membrane translocase subunit Tim23 K17794 TIM23; mitochondrial import inner membrane translocase subunit TIM23
D 100347175 mitochondrial import inner membrane translocase subunit Tim23 isoform X1 K17794 TIM23; mitochondrial import inner membrane translocase subunit TIM23
D 127484182 mitochondrial import inner membrane translocase subunit Tim23 isoform X1 K17794 TIM23; mitochondrial import inner membrane translocase subunit TIM23
D 100345112 mitochondrial import inner membrane translocase subunit Tim17-B isoform X1 K17795 TIM17; mitochondrial import inner membrane translocase subunit TIM17
D 100358025 mitochondrial import inner membrane translocase subunit Tim17-A isoform X1 K17795 TIM17; mitochondrial import inner membrane translocase subunit TIM17
D 100347741 mitochondrial import inner membrane translocase subunit Tim21 K17796 TIM21; mitochondrial import inner membrane translocase subunit TIM21
D 100356545 mitochondrial import inner membrane translocase subunit TIM50 K17496 TIM50; mitochondrial import inner membrane translocase subunit TIM50
C Mitochondrial calcium uniporter complex
D 100341372 calcium uniporter protein, mitochondrial isoform X1 K20858 MCU; calcium uniporter protein, mitochondrial
D 100341636 calcium uptake protein 1, mitochondrial isoform X2 K22827 MICU1; calcium uptake protein 1, mitochondrial
D 100343563 calcium uptake protein 2, mitochondrial K22828 MICU2; calcium uptake protein 2, mitochondrial
D 100358970 calcium uniporter regulatory subunit MCUb, mitochondrial isoform X2 K22829 MCUB; calcium uniporter regulatory subunit MCUb, mitochondrial
D 100349541 essential MCU regulator, mitochondrial isoform X1 K22830 SMDT1; essential MCU regulator, mitochondrial
D 103349686 mitochondrial calcium uniporter regulator 1 K22137 MCUR1; mitochondrial calcium uniporter regulator 1
C Other inner membrane factors
D 100346816 mitochondrial proton/calcium exchanger protein K17800 LETM1; LETM1 and EF-hand domain-containing protein 1, mitochondrial
D 103347528 mitochondrial proton/calcium exchanger protein K17800 LETM1; LETM1 and EF-hand domain-containing protein 1, mitochondrial
D 100358499 mitochondrial inner membrane protease subunit 1 isoform X1 K09647 IMP1; mitochondrial inner membrane protease subunit 1 [EC:3.4.21.-]
D 100354778 mitochondrial inner membrane protease subunit 2 isoform X1 K09648 IMP2; mitochondrial inner membrane protease subunit 2 [EC:3.4.21.-]
D 100344962 transmembrane protein 126A K18157 TMEM126A; transmembrane protein 126A
D 100341642 mitochondrial pyruvate carrier 1-like protein K22138 MPC1; mitochondrial pyruvate carrier 1
D 100344703 mitochondrial pyruvate carrier 1 K22138 MPC1; mitochondrial pyruvate carrier 1
D 100341954 mitochondrial pyruvate carrier 2 K22139 MPC2; mitochondrial pyruvate carrier 2
D 100342475 mitochondrial pyruvate carrier 2 K22139 MPC2; mitochondrial pyruvate carrier 2
D 100347216 mitochondrial pyruvate carrier 2-like K22139 MPC2; mitochondrial pyruvate carrier 2
D 100347734 mitochondrial pyruvate carrier 2-like K22139 MPC2; mitochondrial pyruvate carrier 2
D 127486715 mitochondrial pyruvate carrier 2-like K22139 MPC2; mitochondrial pyruvate carrier 2
D 100350144 coiled-coil-helix-coiled-coil-helix domain-containing protein 10, mitochondrial K22759 CHCHD10; coiled-coil-helix-coiled-coil-helix domain-containing protein 10
B Matrix
C MMC/PAM proteins
D 100353480 mitochondrial import inner membrane translocase subunit TIM44 isoform X1 K17804 TIM44; mitochondrial import inner membrane translocase subunit TIM44
D 100347135 stress-70 protein, mitochondrial K04043 dnaK; molecular chaperone DnaK
D 103351840 LOW QUALITY PROTEIN: stress-70 protein, mitochondrial-like K04043 dnaK; molecular chaperone DnaK
D 100341336 grpE protein homolog 2, mitochondrial isoform X1 K03687 GRPE; molecular chaperone GrpE
D 100354057 LOW QUALITY PROTEIN: grpE protein homolog 2, mitochondrial-like K03687 GRPE; molecular chaperone GrpE
D 127492120 grpE protein homolog 2, mitochondrial-like K03687 GRPE; molecular chaperone GrpE
D 100348759 mitochondrial import inner membrane translocase subunit TIM14 isoform X1 K09539 DNAJC19; DnaJ homolog subfamily C member 19
D 100341390 mitochondrial import inner membrane translocase subunit TIM16 isoform X1 K17805 PAM16; mitochondrial import inner membrane translocase subunit TIM16
C Other matrix factors
D 100340909 mitochondrial-processing peptidase subunit beta isoform X1 K17732 PMPCB; mitochondrial-processing peptidase subunit beta [EC:3.4.24.64]
D 100355371 mitochondrial intermediate peptidase isoform X1 K01410 MIPEP; mitochondrial intermediate peptidase [EC:3.4.24.59]
D 100349912 60 kDa heat shock protein, mitochondrial K04077 groEL; chaperonin GroEL [EC:5.6.1.7]
D 100341867 10 kDa heat shock protein, mitochondrial-like K04078 groES; chaperonin GroES
D 100343795 10 kDa heat shock protein, mitochondrial-like K04078 groES; chaperonin GroES
D 100348970 10 kDa heat shock protein, mitochondrial-like K04078 groES; chaperonin GroES
D 100350401 10 kDa heat shock protein, mitochondrial isoform X4 K04078 groES; chaperonin GroES
D 100350682 10 kDa heat shock protein, mitochondrial-like K04078 groES; chaperonin GroES
D 100350792 10 kDa heat shock protein, mitochondrial-like K04078 groES; chaperonin GroES
D 100353436 10 kDa heat shock protein, mitochondrial-like K04078 groES; chaperonin GroES
D 100356803 10 kDa heat shock protein, mitochondrial K04078 groES; chaperonin GroES
D 100357698 10 kDa heat shock protein, mitochondrial-like K04078 groES; chaperonin GroES
D 127489347 10 kDa heat shock protein, mitochondrial K04078 groES; chaperonin GroES
D 100338697 dnaJ homolog subfamily A member 3, mitochondrial isoform X1 K09504 DNAJA3; DnaJ homolog subfamily A member 3
D 100344662 dnaJ homolog subfamily A member 3, mitochondrial-like isoform X2 K09504 DNAJA3; DnaJ homolog subfamily A member 3
D 100348148 phosphatidate cytidylyltransferase, mitochondrial isoform X2 K17807 TAM41; mitochondrial translocator assembly and maintenance protein 41
D 100340123 persulfide dioxygenase ETHE1, mitochondrial K17725 ETHE1; sulfur dioxygenase [EC:1.13.11.18]
D 127482755 persulfide dioxygenase ETHE1, mitochondrial K17725 ETHE1; sulfur dioxygenase [EC:1.13.11.18]
D 100348174 polymerase delta-interacting protein 2 isoform X1 K17809 POLDIP2; polymerase delta-interacting protein 2
#
AMitochondrial quality control factors
B Regulator of mitochondrial biogenesis
C Ubiquitas transcription factors
D 100351999 cyclic AMP-responsive element-binding protein 1 isoform X1 K05870 CREB1; cyclic AMP-responsive element-binding protein 1
D 100344691 ribosome-releasing factor 2, mitochondrial isoform X1 K02355 fusA; elongation factor G
D 100345180 elongation factor G, mitochondrial K02355 fusA; elongation factor G
D 100351317 transcription factor Sp1 isoform X3 K04684 SP1; transcription factor Sp1
D 103346032 transcriptional repressor protein YY1 K09201 YY; transcription factor YY
D 108178746 transcription factor YY2 K09201 YY; transcription factor YY
D 100352171 GA-binding protein subunit beta-2 isoform X1 K09454 GABPB; GA-binding protein transcription factor, beta
D 100352189 GA-binding protein subunit beta-1 isoform X6 K09454 GABPB; GA-binding protein transcription factor, beta
D 100358902 transcription factor A, mitochondrial K11830 TFAM; transcription factor A, mitochondrial
C Nuclear respiratory factors
D 100339141 nuclear respiratory factor 1 isoform X1 K11831 NRF1; nuclear respiratory factor 1
D 100353014 nuclear factor erythroid 2-related factor 2 isoform X5 K05638 NFE2L2; nuclear factor erythroid 2-related factor 2
D 100343299 metallothionein-2A K14739 MT1_2; metallothionein 1/2
D 100343557 metallothionein-2D K14739 MT1_2; metallothionein 1/2
D 100343802 metallothionein-1A K14739 MT1_2; metallothionein 1/2
D 103350073 metallothionein-II, hippocampal-like K14739 MT1_2; metallothionein 1/2
D 100009025 MT3; metallothionein-3 K14740 MT3; metallothionein 3
D 127491304 metallothionein-4 K14741 MT4; metallothionein 4
C Coactivators
D 100349219 peroxisome proliferator-activated receptor gamma coactivator 1-alpha isoform X1 K07202 PPARGC1A; alpha peroxisome proliferator-activated receptor gamma coactivator 1-alpha
D 100340477 peroxisome proliferator-activated receptor gamma coactivator 1-beta K17962 PPARGC1B; peroxisome proliferator-activated receptor gamma coactivator 1-beta
D 100339175 peroxisome proliferator-activated receptor gamma coactivator-related protein 1 isoform X1 K17963 PPRC1; peroxisome proliferator-activated receptor gamma coactivator-related protein 1
D 100340619 CREB-binding protein isoform X1 K04498 EP300; E1A/CREB-binding protein [EC:2.3.1.48]
D 100354135 histone acetyltransferase p300 isoform X1 K04498 EP300; E1A/CREB-binding protein [EC:2.3.1.48]
C Proteins involved in Fe-S cluster biogenesis
D 100328709 NFS1; cysteine desulfurase, mitochondrial K04487 iscS; cysteine desulfurase [EC:2.8.1.7]
D 100354739 iron-sulfur cluster assembly enzyme ISCU, mitochondrial K22068 ISCU; iron-sulfur cluster assembly enzyme ISCU, mitochondrial
D 100351199 LYR motif-containing protein 4 isoform X2 K22069 LYRM4; LYR motif-containing protein 4
D 103348216 frataxin, mitochondrial isoform X2 K19054 FXN; frataxin [EC:1.16.3.1]
D 100352891 adrenodoxin, mitochondrial isoform X1 K22070 FDX1; ferredoxin-1, mitochondrial
D 127488504 ferredoxin-2, mitochondrial K22071 FDX2; ferredoxin-2, mitochondrial
D 103346270 NADPH:adrenodoxin oxidoreductase, mitochondrial K18914 FDXR; adrenodoxin-NADP+ reductase [EC:1.18.1.6]
D 100347135 stress-70 protein, mitochondrial K04043 dnaK; molecular chaperone DnaK
D 103351840 LOW QUALITY PROTEIN: stress-70 protein, mitochondrial-like K04043 dnaK; molecular chaperone DnaK
D 100355237 iron-sulfur cluster co-chaperone protein HscB isoform X1 K04082 hscB; molecular chaperone HscB
D 100347643 nucleotide exchange factor SIL1 isoform X1 K14001 SIL1; nucleotide exchange factor SIL1
D 100352734 glutaredoxin-related protein 5, mitochondrial K07390 grxD; monothiol glutaredoxin
D 100342257 iron-sulfur cluster assembly 1 homolog, mitochondrial K22063 ISCA1; iron-sulfur cluster assembly 1
D 100340528 iron-sulfur cluster assembly 2 homolog, mitochondrial isoform X2 K22072 ISCA2; iron-sulfur cluster assembly 2
D 100354839 putative transferase CAF17, mitochondrial isoform X1 K22073 IBA57; transferase CAF17, mitochondrial [EC:2.1.-.-]
D 100357378 NFU1 iron-sulfur cluster scaffold homolog, mitochondrial isoform X2 K22074 NFU1; NFU1 iron-sulfur cluster scaffold homolog, mitochondrial
D 100357656 bolA-like protein 1 K22066 BOLA1; BolA-like protein 1
D 100340875 bolA-like protein 3 K22075 BOLA3; BolA-like protein 3
D 100349901 bolA-like protein 3 K22075 BOLA3; BolA-like protein 3
D 100344214 sideroflexin-1 isoform X1 K23500 SFXN1_3; sideroflexin-1/3
D 100349780 sideroflexin-3 isoform X1 K23500 SFXN1_3; sideroflexin-1/3
D 100345875 sideroflexin-2 isoform X5 K23501 SFXN2; sideroflexin-2
D 103351437 sideroflexin-4 isoform X1 K23502 SFXN4; sideroflexin-4
D 100358331 sideroflexin-5 isoform X1 K23503 SFXN5; sideroflexin-5
C Other regulator of mitochondrial biogenesis
D 100350311 carnitine O-palmitoyltransferase 1, liver isoform K08765 CPT1A; carnitine O-palmitoyltransferase 1, liver isoform [EC:2.3.1.21]
D 100350716 carnitine O-palmitoyltransferase 1, liver isoform isoform X1 K08765 CPT1A; carnitine O-palmitoyltransferase 1, liver isoform [EC:2.3.1.21]
D 103346593 carnitine O-palmitoyltransferase 1, liver isoform isoform X1 K08765 CPT1A; carnitine O-palmitoyltransferase 1, liver isoform [EC:2.3.1.21]
D 100353138 carnitine O-palmitoyltransferase 1, muscle isoform isoform X1 K19523 CPT1B; carnitine O-palmitoyltransferase 1, muscle isoform [EC:2.3.1.21]
D 100341327 carnitine O-palmitoyltransferase 1, brain isoform K19524 CPT1C; carnitine O-palmitoyltransferase 1, brain isoform [EC:2.3.1.21]
D 100356735 2-oxoadipate dehydrogenase complex component E1 K15791 DHKTD1; 2-oxoadipate dehydrogenase E1 component [EC:1.2.4.-]
D 100344579 F-box/LRR-repeat protein 4 K10270 FBXL4; F-box and leucine-rich repeat protein 4
D 100352051 host cell factor 1 isoform X6 K14966 HCFC1; host cell factor 1
D 100343128 host cell factor 2 isoform X6 K27390 HCFC2; host cell factor 2
D 100351801 huntingtin K04533 HD; huntingtin
D 103347399 steroid hormone receptor ERR1 K08552 NR3B1; estrogen-related receptor alpha
D 100338685 steroid hormone receptor ERR2 isoform X1 K08553 NR3B2; estrogen-related receptor beta
D 100355568 estrogen-related receptor gamma isoform X1 K08554 NR3B3; estrogen-related receptor gamma
D 100348085 nuclear receptor-interacting protein 1 K17965 NRIP1; nuclear receptor-interacting protein 1
D 100340670 5'-AMP-activated protein kinase catalytic subunit alpha-2 isoform X2 K07198 PRKAA; 5'-AMP-activated protein kinase, catalytic alpha subunit [EC:2.7.11.31]
D 100358166 5'-AMP-activated protein kinase catalytic subunit alpha-1 K07198 PRKAA; 5'-AMP-activated protein kinase, catalytic alpha subunit [EC:2.7.11.31]
D 100349271 NAD-dependent protein deacetylase sirtuin-1 isoform X1 K11411 SIRT1; NAD-dependent protein deacetylase sirtuin 1 [EC:2.3.1.286]
D 100338574 protein SERAC1 isoform X1 K23504 SERAC1; protein SERAC1
D 100341210 protein UXT isoform X2 K26537 UXT; protein UXT
D 100354759 PGC-1 and ERR-induced regulator in muscle protein 1 K26738 PERM1; PGC-1 and ERR-induced regulator in muscle protein 1
D 127485849 putative glutamine amidotransferase-like class 1 domain-containing protein 3B, mitochondrial 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 100348510 complex I intermediate-associated protein 30, mitochondrial K18159 NDUFAF1; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex assembly factor 1
D 100353024 NADH dehydrogenase [ubiquinone] 1 alpha subcomplex assembly factor 2 isoform X6 K18160 NDUFAF2; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex assembly factor 2
D 100355126 NADH dehydrogenase [ubiquinone] 1 alpha subcomplex assembly factor 3 K09008 NDUFAF3; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex assembly factor 3
D 100356197 NADH dehydrogenase [ubiquinone] 1 alpha subcomplex assembly factor 4 K18161 NDUFAF4; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex assembly factor 4
D 100337968 arginine-hydroxylase NDUFAF5, mitochondrial isoform X1 K18162 NDUFAF5; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex assembly factor 5 [EC:2.1.1.-]
D 100358419 NADH dehydrogenase (ubiquinone) complex I, assembly factor 6 isoform X1 K18163 NDUFAF6; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex assembly factor 6
D 100338468 protein arginine methyltransferase NDUFAF7, mitochondrial isoform X1 K18164 NDUFAF7; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex assembly factor 7
D 100344587 protein arginine methyltransferase NDUFAF7, mitochondrial-like K18164 NDUFAF7; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex assembly factor 7
D 127489969 NADH dehydrogenase [ubiquinone] 1 alpha subcomplex assembly factor 8 K24726 NDUFAF8; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex assembly factor 8
D 100343876 complex I assembly factor TMEM126B, mitochondrial-like K18165 TMEM126B; complex I assembly factor TMEM126B
D 100345213 complex I assembly factor TMEM126B, mitochondrial isoform X2 K18165 TMEM126B; complex I assembly factor TMEM126B
D 100355385 complex I assembly factor TMEM126B, mitochondrial-like K18165 TMEM126B; complex I assembly factor TMEM126B
D 100355017 evolutionarily conserved signaling intermediate in Toll pathway, mitochondrial isoform X2 K04405 ECSIT; evolutionarily conserved signaling intermediate in Toll pathway
D 100343898 complex I assembly factor ACAD9, mitochondrial K15980 ACAD9; acyl-CoA dehydrogenase family member 9 [EC:1.3.99.-]
D 100358634 iron-sulfur protein NUBPL isoform X4 K03593 mrp; ATP-binding protein involved in chromosome partitioning
D 100338957 FAD-dependent oxidoreductase domain-containing protein 1 isoform X2 K18166 FOXRED1; FAD-dependent oxidoreductase domain-containing protein 1
D 100338994 reticulon-4-interacting protein 1, mitochondrial K23164 RTN4IP1; reticulon-4-interacting protein 1, mitochondrial
D 100355713 complex I assembly factor TIMMDC1, mitochondrial isoform X1 K23505 TIMMDC1; complex I assembly factor TIMMDC1
C Complex-II assembly factors
D 100348442 succinate dehydrogenase assembly factor 1, mitochondrial K18167 SDHAF1; succinate dehydrogenase assembly factor 1
D 100353725 succinate dehydrogenase assembly factor 2, mitochondrial K18168 SDHAF2; succinate dehydrogenase assembly factor 2
C Complex-III assembly factors
D 100351828 mitochondrial chaperone BCS1 K08900 BCS1; mitochondrial chaperone BCS1
D 100354061 tetratricopeptide repeat protein 19, mitochondrial isoform X1 K18169 TTC19; tetratricopeptide repeat protein 19, mitochondrial
D 100352906 complex III assembly factor LYRM7 isoform X1 K18170 LYRM7; complex III assembly factor LYRM7
D 100355688 ubiquinol-cytochrome-c reductase complex assembly factor 1 isoform X1 K17662 CBP3; cytochrome b pre-mRNA-processing protein 3
D 100355204 ubiquinol-cytochrome-c reductase complex assembly factor 2 K17682 MNF1; mitochondrial nucleoid factor 1
D 100349105 ubiquinol-cytochrome-c reductase complex assembly factor 3 K23165 UQCC3; ubiquinol-cytochrome-c reductase complex assembly factor 3
D 100345570 LOW QUALITY PROTEIN: protein CCSMST1 K27339 UQCC4; ubiquinol-cytochrome c reductase complex assembly factor 4
D 127486413 protein CCSMST1 K27339 UQCC4; ubiquinol-cytochrome c reductase complex assembly factor 4
D 100347316 small integral membrane protein 4 K27340 UQCC5; ubiquinol-cytochrome c reductase complex assembly factor 5
D 100342621 protein BRAWNIN isoform X6 K27341 UQCC6; ubiquinol-cytochrome c reductase complex assembly factor 6
D 103348648 STMP1; short transmembrane mitochondrial protein 1 isoform X2 K27319 STMP1; short transmembrane mitochondrial protein 1
C Complex-IV assembly factors
D 100355389 COX assembly mitochondrial protein homolog isoform X1 K18171 CMC1; COX assembly mitochondrial protein 1
D 127487668 COX assembly mitochondrial protein homolog K18171 CMC1; COX assembly mitochondrial protein 1
D 100339139 COX assembly mitochondrial protein 2 homolog isoform X4 K18172 CMC2; COX assembly mitochondrial protein 2
D 100352100 cytochrome c oxidase assembly factor 3 homolog, mitochondrial K18175 CCDC56; cytochrome c oxidase assembly factor 3, animal type
D 100357033 cytochrome c oxidase assembly factor 4 homolog, mitochondrial K18177 COA4; cytochrome c oxidase assembly factor 4
D 100340218 cytochrome c oxidase assembly factor 5 K18178 COA5; cytochrome c oxidase assembly factor 5
D 100354303 cytochrome c oxidase assembly factor 6 homolog K18179 COA6; cytochrome c oxidase assembly factor 6
D 100355134 cytochrome c oxidase assembly factor 7 K18180 COA7; cytochrome c oxidase assembly factor 7
D 100356833 protoheme IX farnesyltransferase, mitochondrial K02257 COX10; heme o synthase [EC:2.5.1.141]
D 100338343 cytochrome c oxidase assembly protein COX11, mitochondrial K02258 COX11; cytochrome c oxidase assembly protein subunit 11
D 100339718 cytochrome c oxidase assembly protein COX14 K18181 COX14; cytochrome c oxidase assembly factor 14
D 100355645 cytochrome c oxidase assembly protein COX15 homolog K02259 COX15; heme a synthase [EC:1.17.99.9]
D 100339352 LOW QUALITY PROTEIN: cytochrome c oxidase assembly protein COX16 homolog, mitochondrial K18182 COX16; cytochrome c oxidase assembly protein subunit 16
D 100352275 cytochrome c oxidase copper chaperone K02260 COX17; cytochrome c oxidase assembly protein subunit 17
D 100355311 cytochrome c oxidase assembly protein COX18, mitochondrial isoform X2 K17797 COX18; mitochondrial inner membrane protein COX18
D 100349428 cytochrome c oxidase assembly protein COX20, mitochondrial isoform X3 K18184 COX20; cytochrome c oxidase assembly protein subunit 20
D 100354547 LOW QUALITY PROTEIN: cytochrome c oxidase assembly protein COX20, mitochondrial-like K18184 COX20; cytochrome c oxidase assembly protein subunit 20
D 100340355 protein PET100 homolog, mitochondrial K18186 PET100; protein PET100, animal type
D 100356541 protein PET117 homolog, mitochondrial K18188 PET117; protein PET117
D 100343588 mitochondrial inner membrane protein OXA1L K03217 yidC; YidC/Oxa1 family membrane protein insertase
D 100338010 protein SCO1 homolog, mitochondrial K07152 SCO1; protein SCO1
D 100348091 translational activator of cytochrome c oxidase 1 K18189 TACO1; translational activator of cytochrome c oxidase 1
D 100357043 leucine-rich PPR motif-containing protein, mitochondrial K17964 LRPPRC; leucine-rich PPR motif-containing protein, mitochondrial
D 103348648 STMP1; short transmembrane mitochondrial protein 1 isoform X2 K27319 STMP1; short transmembrane mitochondrial protein 1
D 100347316 small integral membrane protein 4 K27340 UQCC5; ubiquinol-cytochrome c reductase complex assembly factor 5
D 100338601 transmembrane protein 223 K27342 TMEM223; transmembrane protein 223
C Complex-V assembly factors
D 100342302 ATP synthase mitochondrial F1 complex assembly factor 1 isoform X1 K07555 ATPeAF1; ATP synthase mitochondrial F1 complex assembly factor 1
D 100353151 ATP synthase mitochondrial F1 complex assembly factor 2 isoform X4 K07556 ATPeAF2; ATP synthase mitochondrial F1 complex assembly factor 2
D 100353330 ATP synthase mitochondrial F1 complex assembly factor 2 isoform X1 K07556 ATPeAF2; ATP synthase mitochondrial F1 complex assembly factor 2
D 108175430 LOW QUALITY PROTEIN: ATP synthase mitochondrial F1 complex assembly factor 2 K07556 ATPeAF2; ATP synthase mitochondrial F1 complex assembly factor 2
D 100341511 transmembrane protein 70, mitochondrial K17966 TMEM70; transmembrane protein 70, mitochondrial
D 100339431 ATP5MJ; ATP synthase subunit ATP5MJ, mitochondrial K18193 MP68; 6.8 kDa mitochondrial proteolipid
D 108176918 ATP synthase membrane subunit K, mitochondrial-like K18194 USMG5; up-regulated during skeletal muscle growth protein 5
B Mitochondrial dynamics
C Fission and Fusion factors
D 100346474 mitofusin-1 isoform X1 K21356 MFN1; mitofusin 1 [EC:3.6.5.-]
D 100350119 mitofusin-2 K06030 MFN2; mitofusin 2 [EC:3.6.5.-]
D 100344494 dynamin-like 120 kDa protein, mitochondrial isoform X2 K17079 OPA1; optic atrophy protein 1 [EC:3.6.5.5]
D 100349723 optic atrophy 3 protein homolog K23166 OPA3; optic atrophy 3 protein
D 100349976 optic atrophy 3 protein K23166 OPA3; optic atrophy 3 protein
D 108178657 TP53-regulated inhibitor of apoptosis 1 K17968 TRIAP1; TRIAP1/MDM35 family protein
D 127485150 TP53-regulated inhibitor of apoptosis 1-like K17968 TRIAP1; TRIAP1/MDM35 family protein
D 100351338 presenilins-associated rhomboid-like protein, mitochondrial isoform X1 K09650 PARL; rhomboid-like protein [EC:3.4.21.105]
D 100351759 LOW QUALITY PROTEIN: presenilins-associated rhomboid-like protein, mitochondrial K09650 PARL; rhomboid-like protein [EC:3.4.21.105]
D 100342046 bcl-2 homologous antagonist/killer K14021 BAK; Bcl-2 homologous antagonist/killer
D 100355675 apoptosis regulator BAX isoform X2 K02159 BAX; apoptosis regulator BAX
D 100338814 dynamin-1-like protein isoform X4 K17065 DNM1L; dynamin 1-like protein [EC:3.6.5.5]
D 100341062 mitochondrial fission 1 protein K17969 FIS1; mitochondrial fission 1 protein
D 100340132 small ubiquitin-related modifier 2 K12160 SUMO; small ubiquitin-related modifier
D 100342981 small ubiquitin-related modifier 2-like K12160 SUMO; small ubiquitin-related modifier
D 100343020 small ubiquitin-related modifier 3 K12160 SUMO; small ubiquitin-related modifier
D 100344119 small ubiquitin-related modifier 2 isoform X2 K12160 SUMO; small ubiquitin-related modifier
D 100344452 small ubiquitin-related modifier 2-like K12160 SUMO; small ubiquitin-related modifier
D 100345593 small ubiquitin-related modifier 2-like K12160 SUMO; small ubiquitin-related modifier
D 100345604 small ubiquitin-related modifier 1-like K12160 SUMO; small ubiquitin-related modifier
D 100350000 small ubiquitin-related modifier 1 isoform X3 K12160 SUMO; small ubiquitin-related modifier
D 100353123 small ubiquitin-related modifier 1-like K12160 SUMO; small ubiquitin-related modifier
D 100354027 small ubiquitin-related modifier 2 K12160 SUMO; small ubiquitin-related modifier
D 100354994 small ubiquitin-related modifier 3-like K12160 SUMO; small ubiquitin-related modifier
D 127483781 small ubiquitin-related modifier 2-like K12160 SUMO; small ubiquitin-related modifier
D 127491578 small ubiquitin-related modifier 2-like K12160 SUMO; small ubiquitin-related modifier
D 100352421 endophilin-B1 isoform X6 K11248 SH3GLB1; endophilin-B1
D 127488183 endophilin-B1 isoform X1 K11248 SH3GLB1; endophilin-B1
D 100355843 mitochondrial carrier homolog 2 isoform X1 K17885 MTCH; mitochondrial carrier
D 103349752 mitochondrial carrier homolog 1 K17885 MTCH; mitochondrial carrier
D 100340489 mitochondrial fission factor isoform X2 K22076 MFF; mitochondrial fission factor
D 100356618 mitochondrial fission factor-like K22076 MFF; mitochondrial fission factor
D 100342027 ganglioside-induced differentiation-associated protein 1 isoform X1 K22077 GDAP1; ganglioside-induced differentiation-associated protein 1
D 100347940 ganglioside-induced differentiation-associated protein 1-like 1 isoform X2 K22077 GDAP1; ganglioside-induced differentiation-associated protein 1
D 100347006 synaptic vesicle membrane protein VAT-1 homolog K23167 VAT1; synaptic vesicle membrane protein VAT-1
D 100349916 LOW QUALITY PROTEIN: mitochondrial dynamics protein MID51-like K23507 MIEF1_2; mitochondrial dynamics protein MID51/49
D 100357986 mitochondrial dynamics protein MID51 K23507 MIEF1_2; mitochondrial dynamics protein MID51/49
D 103346090 mitochondrial cardiolipin hydrolase K16862 PLD6; mitochondrial cardiolipin hydrolase [EC:3.1.4.-]
D 100345140 mitoguardin 2 K27289 MIGA; mitoguardin
D 100345951 mitoguardin 1 isoform X1 K27289 MIGA; mitoguardin
C Actin cytoskeleton organization
D 100008822 actin, cytoplasmic 1 K05692 ACTB_G1; actin beta/gamma 1
D 100009272 ACTB; actin, cytoplasmic 1 K05692 ACTB_G1; actin beta/gamma 1
D 100341037 LOW QUALITY PROTEIN: actin, cytoplasmic 2-like K05692 ACTB_G1; actin beta/gamma 1
D 100353912 N-alpha-acetyltransferase 20 K17972 NAA20; N-terminal acetyltransferase B complex catalytic subunit [EC:2.3.1.254]
D 100354816 N-alpha-acetyltransferase 25, NatB auxiliary subunit isoform X1 K17973 NAA25; N-terminal acetyltransferase B complex non-catalytic subunit
C Mitochondrial movement
D 100344182 PHB1; prohibitin 1 K17080 PHB1; prohibitin 1
D 100341779 prohibitin-2 K17081 PHB2; prohibitin 2
D 100358191 kinectin isoform X1 K17975 KTN1; kinectin
C Retention of mitochondria
D 100338814 dynamin-1-like protein isoform X4 K17065 DNM1L; dynamin 1-like protein [EC:3.6.5.5]
D 100008736 unconventional myosin-Vb K10357 MYO5; myosin V
D 100346225 unconventional myosin-Va isoform X1 K10357 MYO5; myosin V
D 100346740 unconventional myosin-Vc K10357 MYO5; myosin V
D 100328700 XIAP; E3 ubiquitin-protein ligase XIAP K04725 XIAP; E3 ubiquitin-protein ligase XIAP [EC:2.3.2.27]
D 100345253 E3 ubiquitin-protein ligase XIAP-like K04725 XIAP; E3 ubiquitin-protein ligase XIAP [EC:2.3.2.27]
D 100344529 diablo IAP-binding mitochondrial protein isoform X1 K10522 DIABLO; diablo
C Morphogenesis factors
D 103345646 sorting nexin-25 isoform X1 K17887 SNX25; sorting nexin-25
D 100351349 mitochondrial Rho GTPase 1 isoform X3 K07870 RHOT1; mitochondrial Rho GTPase 1 [EC:3.6.5.-]
D 100337704 MICOS complex subunit MIC60 isoform X1 K17785 IMMT; MICOS complex subunit MIC60
D 100354379 mitochondrial fission process protein 1 isoform X1 K17981 MTFP1; mitochondrial fission process protein 1
D 100353283 28S ribosomal protein S29, mitochondrial isoform X1 K17408 DAP3; small subunit ribosomal protein S29
D 100009418 E3 ubiquitin-protein ligase NEDD4 isoform X1 K10591 NEDD4; E3 ubiquitin-protein ligase NEDD4 [EC:2.3.2.26]
D 100345410 oxidation resistance protein 1 isoform X6 K25437 OXR1; oxidation resistance protein 1
D 100352018 protein misato homolog 1 isoform X1 K27320 MSTO1; protein misato
D 100356619 PRELI domain-containing protein 1, mitochondrial isoform X2 K27964 PRELID1; PRELI domain-containing protein 1, mitochondrial
D 100356536 PRELI domain-containing protein 2 K27965 PRELID2; PRELI domain-containing protein 2 mitochondrial
D 100348105 PRELI domain containing protein 3B K27966 PRELID3; PRELI domain-containing protein 3, mitochondrial
D 103349287 PRELI domain containing protein 3A isoform X3 K27966 PRELID3; PRELI domain-containing protein 3, mitochondrial
B Mitophagy factors
C Autophagy-related proteins
D 100339862 autophagy-related protein 2 homolog B K17906 ATG2; autophagy-related protein 2
D 103346157 autophagy-related protein 2 homolog A isoform X1 K17906 ATG2; autophagy-related protein 2
D 100348916 ubiquitin-like-conjugating enzyme ATG3 K08343 ATG3; ubiquitin-like-conjugating enzyme ATG3
D 100338183 cysteine protease ATG4A isoform X1 K08342 ATG4; cysteine protease ATG4 [EC:3.4.22.-]
D 100342766 cysteine protease ATG4B isoform X3 K08342 ATG4; cysteine protease ATG4 [EC:3.4.22.-]
D 100349970 cysteine protease ATG4D K08342 ATG4; cysteine protease ATG4 [EC:3.4.22.-]
D 100357847 cysteine protease ATG4C isoform X1 K08342 ATG4; cysteine protease ATG4 [EC:3.4.22.-]
D 100342807 autophagy protein 5 isoform X2 K08339 ATG5; autophagy-related protein 5
D 100344446 LOW QUALITY PROTEIN: beclin-1-like K08334 BECN; beclin
D 100352353 beclin-1 isoform X1 K08334 BECN; beclin
D 100354712 beclin-2 K08334 BECN; beclin
D 100342374 ubiquitin-like modifier-activating enzyme ATG7 isoform X1 K08337 ATG7; ubiquitin-like modifier-activating enzyme ATG7
D 100008883 GABARAP; gamma-aminobutyric acid receptor-associated protein K08341 GABARAP; GABA(A) receptor-associated protein
D 100340751 gamma-aminobutyric acid receptor-associated protein-like 1 K08341 GABARAP; GABA(A) receptor-associated protein
D 100342698 gamma-aminobutyric acid receptor-associated protein-like 2 isoform X2 K08341 GABARAP; GABA(A) receptor-associated protein
D 103349871 gamma-aminobutyric acid receptor-associated protein-like K08341 GABARAP; GABA(A) receptor-associated protein
D 100350757 autophagy-related protein 9B K17907 ATG9; autophagy-related protein 9
D 100350996 autophagy-related protein 9A isoform X1 K17907 ATG9; autophagy-related protein 9
D 127488309 autophagy-related protein 9B K17907 ATG9; autophagy-related protein 9
D 103349534 ubiquitin-like-conjugating enzyme ATG10 isoform X3 K17888 ATG10L; ubiquitin-like-conjugating enzyme ATG10
D 100346703 ubiquitin-like protein ATG12 K08336 ATG12; ubiquitin-like protein ATG12
D 103347845 ubiquitin-like protein ATG12 isoform X1 K08336 ATG12; ubiquitin-like protein ATG12
D 100357931 beclin 1-associated autophagy-related key regulator K17889 ATG14L; beclin 1-associated autophagy-related key regulator
D 100345122 autophagy-related protein 16-1 isoform X2 K17890 ATG16L1; autophagy-related protein 16-1
D 100359102 protein Atg16l2 isoform X1 K20868 ATG16L2; autophagy-related protein 16-2
D 100340527 WD repeat domain phosphoinositide-interacting protein 1 K17908 WIPI1_2; autophagy-related protein 18
D 100352881 WD repeat domain phosphoinositide-interacting protein 2 isoform X1 K17908 WIPI1_2; autophagy-related protein 18
C Parkin-dependent mechanism factors
D 100358886 PRKN; E3 ubiquitin-protein ligase parkin isoform X1 K04556 PARK2; parkin [EC:2.3.2.31]
D 100350071 E3 ubiquitin-protein ligase NRDP1 K11981 RNF41; E3 ubiquitin-protein ligase NRDP1 [EC:2.3.2.27]
D 127487395 E3 ubiquitin-protein ligase NRDP1 K11981 RNF41; E3 ubiquitin-protein ligase NRDP1 [EC:2.3.2.27]
D 100339140 protein CLEC16A isoform X1 K19513 CLEC16A; protein CLEC16A
D 100350666 serine/threonine-protein kinase PINK1, mitochondrial K05688 PINK1; PTEN induced putative kinase 1 [EC:2.7.11.1]
D 100351338 presenilins-associated rhomboid-like protein, mitochondrial isoform X1 K09650 PARL; rhomboid-like protein [EC:3.4.21.105]
D 100351759 LOW QUALITY PROTEIN: presenilins-associated rhomboid-like protein, mitochondrial K09650 PARL; rhomboid-like protein [EC:3.4.21.105]
D 100342157 sequestosome-1 isoform X1 K14381 SQSTM1; sequestosome 1
D 100343901 tax1-binding protein 1 isoform X2 K21347 TAX1BP1; Tax1-binding protein 1
D 100351096 calcium-binding and coiled-coil domain-containing protein 2 isoform X3 K21348 CALCOCO2; calcium-binding and coiled-coil domain-containing protein 2
D 100349558 activating molecule in BECN1-regulated autophagy protein 1 isoform X1 K17985 AMBRA1; activating molecule in BECN1-regulated autophagy protein 1
C Parkin-independent mechanism factors
D 100350226 autophagy-related protein 13 isoform X6 K08331 ATG13; autophagy-related protein 13
D 100350803 serine/threonine-protein kinase ULK1 isoform X1 K21357 ULK1; serine/threonine-protein kinase ULK1 [EC:2.7.11.1]
D 100353118 serine/threonine-protein kinase ULK2 isoform X2 K08269 ULK2; serine/threonine-protein kinase ULK2 [EC:2.7.11.1]
D 100351898 RB1-inducible coiled-coil protein 1 isoform X1 K17589 RB1CC1; RB1-inducible coiled-coil protein 1
D 100338694 heat shock protein HSP 90-alpha K04079 HSP90A; molecular chaperone HtpG
D 100358690 heat shock protein HSP 90-beta K04079 HSP90A; molecular chaperone HtpG
D 100344117 hsp90 co-chaperone Cdc37 K09554 CDC37; cell division cycle protein 37
D 100353573 E3 ubiquitin-protein ligase SMURF1 isoform X1 K04678 SMURF; E3 ubiquitin ligase SMURF1/2 [EC:2.3.2.26]
D 100358121 E3 ubiquitin-protein ligase SMURF2 isoform X1 K04678 SMURF; E3 ubiquitin ligase SMURF1/2 [EC:2.3.2.26]
D 100351388 BCL2/adenovirus E1B 19 kDa protein-interacting protein 3-like isoform X1 K15465 BNIP3L; BCL2/adenovirus E1B 19 kDa protein-interacting protein 3-like
D 100347410 FUN14 domain-containing protein 1 K17986 FUNDC1; FUN14 domain-containing protein 1
D 100359077 FUN14 domain-containing protein 1 isoform X1 K17986 FUNDC1; FUN14 domain-containing protein 1
C Other mitophagy factors
D 100340670 5'-AMP-activated protein kinase catalytic subunit alpha-2 isoform X2 K07198 PRKAA; 5'-AMP-activated protein kinase, catalytic alpha subunit [EC:2.7.11.31]
D 100358166 5'-AMP-activated protein kinase catalytic subunit alpha-1 K07198 PRKAA; 5'-AMP-activated protein kinase, catalytic alpha subunit [EC:2.7.11.31]
D 100349465 microtubule-associated proteins 1A/1B light chain 3B isoform X1 K10435 MAP1LC; microtubule-associated protein 1 light chain
D 100349519 microtubule-associated proteins 1A/1B light chain 3C K10435 MAP1LC; microtubule-associated protein 1 light chain
D 127491124 microtubule-associated proteins 1A/1B light chain 3A K10435 MAP1LC; microtubule-associated protein 1 light chain
D 100354979 next to BRCA1 gene 1 protein isoform X1 K17987 NBR1; next to BRCA1 gene 1 protein
D 100356513 tectonin beta-propeller repeat-containing protein 1 isoform X1 K17988 TECPR1; tectonin beta-propeller repeat-containing protein 1
D 100346566 zinc finger FYVE domain-containing protein 1 isoform X2 K17603 ZFYVE1; zinc finger FYVE domain-containing protein 1
D 127482663 LOW QUALITY PROTEIN: zinc finger FYVE domain-containing protein 1-like K17603 ZFYVE1; zinc finger FYVE domain-containing protein 1
D 100344683 bifunctional coenzyme A synthase K02318 COASY; phosphopantetheine adenylyltransferase / dephospho-CoA kinase [EC:2.7.7.3 2.7.1.24]
D 100008961 PLA2G6; 85/88 kDa calcium-independent phospholipase A2 K16343 PLA2G6; calcium-independent phospholipase A2 [EC:3.1.1.4]
D 127490931 85/88 kDa calcium-independent phospholipase A2-like K16343 PLA2G6; calcium-independent phospholipase A2 [EC:3.1.1.4]
D 100352044 CUNH19orf12; protein C19orf12 homolog isoform X1 K23168 MPAN; protein MPAN
D 100328769 FUNDC2; FUN14 domain-containing protein 2 K24308 FUNDC2; FUN14 domain-containing protein 2
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#[ BRITE | KEGG2 | KEGG ]
#Last updated: December 3, 2024