Mitochondrial respiratory chain complex [mitocomplex]
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1. Mitochondrial complex I: NADH dehydrogenase (ubiquinone) subunits
| Approved Symbol | Approved Name | Previous Symbols | Synonyms | Chromosome |
|---|---|---|---|---|
| MT-ND1 | mitochondrially encoded NADH dehydrogenase 1 | MTND1 | ND1, NAD1 | mitochondria |
| MT-ND2 | mitochondrially encoded NADH dehydrogenase 2 | MTND2 | ND2, NAD2 | mitochondria |
| MT-ND3 | mitochondrially encoded NADH dehydrogenase 3 | MTND3 | ND3, NAD3 | mitochondria |
| MT-ND4 | mitochondrially encoded NADH dehydrogenase 4 | MTND4 | ND4, NAD4 | mitochondria |
| MT-ND4L | mitochondrially encoded NADH dehydrogenase 4L | MTND4L | ND4L, NAD4L | mitochondria |
| MT-ND5 | mitochondrially encoded NADH dehydrogenase 5 | MTND5 | ND5, NAD5 | mitochondria |
| MT-ND6 | mitochondrially encoded NADH dehydrogenase 6 | MTND6 | NAD6, ND6 | mitochondria |
| NDUFA1 | NADH dehydrogenase (ubiquinone) 1 alpha subcomplex, 1, 7.5kDa | MWFE, CI-MWFE | Xq24 | |
| NDUFA2 | NADH dehydrogenase (ubiquinone) 1 alpha subcomplex, 2, 8kDa | B8 | 5q31.2 | |
| NDUFA3 | NADH dehydrogenase (ubiquinone) 1 alpha subcomplex, 3, 9kDa | B9 | 19q13.42 | |
| NDUFA4 | NADH dehydrogenase (ubiquinone) 1 alpha subcomplex, 4, 9kDa | MLRQ, CI-9k | 7p21.3 | |
| NDUFA5 | NADH dehydrogenase (ubiquinone) 1 alpha subcomplex, 5, 13kDa | B13, NUFM, CI-13KD-B, UQOR13, CI-13kB | 7q31.33 | |
| NDUFA6 | NADH dehydrogenase (ubiquinone) 1 alpha subcomplex, 6, 14kDa | B14, LYRM6, CI-B14, NADHB14 | 22q13.2 | |
| NDUFA7 | NADH dehydrogenase (ubiquinone) 1 alpha subcomplex, 7, 14.5kDa | B14.5a | 19p13.2 | |
| NDUFA8 | NADH dehydrogenase (ubiquinone) 1 alpha subcomplex, 8, 19kDa | PGIV, MGC793 | 9q33.2 | |
| NDUFA9 | NADH dehydrogenase (ubiquinone) 1 alpha subcomplex, 9, 39kDa | NDUFS2L | SDR22E1, CI-39k | 12p13.3 |
| NDUFA10 | NADH dehydrogenase (ubiquinone) 1 alpha subcomplex, 10, 42kDa | CI-42k | 2q37.3 | |
| NDUFA11 | NADH dehydrogenase (ubiquinone) 1 alpha subcomplex, 11, 14.7kDa | B14.7 | 19p13.3 | |
| NDUFA12 | NADH dehydrogenase (ubiquinone) 1 alpha subcomplex, 12 | DAP13, B17.2 | 12q22 | |
| NDUFA13 | NADH dehydrogenase (ubiquinone) 1 alpha subcomplex, 13 | CGI-39, CDA016, GRIM-19, GRIM19, B16.6 | 19p13.11 | |
| NDUFAB1 | NADH dehydrogenase (ubiquinone) 1, alpha/beta subcomplex, 1, 8kDa | SDAP, FASN2A, ACP | 16p12.3 | |
| NDUFB1 | NADH dehydrogenase (ubiquinone) 1 beta subcomplex, 1, 7kDa | MNLL, CI-MNLL | 14q31.3 | |
| NDUFB2 | NADH dehydrogenase (ubiquinone) 1 beta subcomplex, 2, 8kDa | AGGG, CI-AGGG | 7q34 | |
| NDUFB3 | NADH dehydrogenase (ubiquinone) 1 beta subcomplex, 3, 12kDa | B12 | 2q33.1 | |
| NDUFB4 | NADH dehydrogenase (ubiquinone) 1 beta subcomplex, 4, 15kDa | B15 | 3q13.33 | |
| NDUFB5 | NADH dehydrogenase (ubiquinone) 1 beta subcomplex, 5, 16kDa | SGDH, CI-SGDH, MGC12314 | 3q27.1 | |
| NDUFB6 | NADH dehydrogenase (ubiquinone) 1 beta subcomplex, 6, 17kDa | B17, CI | 9p13.2 | |
| NDUFB7 | NADH dehydrogenase (ubiquinone) 1 beta subcomplex, 7, 18kDa | B18, CI-B18, MGC2480 | 19p13.12 | |
| NDUFB8 | NADH dehydrogenase (ubiquinone) 1 beta subcomplex, 8, 19kDa | ASHI, CI-ASHI | 10q24.31 | |
| NDUFB9 | NADH dehydrogenase (ubiquinone) 1 beta subcomplex, 9, 22kDa | B22, UQOR22, LYRM3 | 8q24.13 | |
| NDUFB10 | NADH dehydrogenase (ubiquinone) 1 beta subcomplex, 10, 22kDa | PDSW | 16p13.3 | |
| NDUFB11 | NADH dehydrogenase (ubiquinone) 1 beta subcomplex, 11, 17.3kDa | ESSS, NP17.3, Np15 | Xp11.3 | |
| NDUFC1 | NADH dehydrogenase (ubiquinone) 1, subcomplex unknown, 1, 6kDa | KFYI | 4q31.1 | |
| NDUFC2 | NADH dehydrogenase (ubiquinone) 1, subcomplex unknown, 2, 14.5kDa | B14.5b, HLC-1 | 11q14.1 | |
| NDUFS1 | NADH dehydrogenase (ubiquinone) Fe-S protein 1, 75kDa (NADH-coenzyme Q reductase) | CI-75k | 2q33-q34 | |
| NDUFS2 | NADH dehydrogenase (ubiquinone) Fe-S protein 2, 49kDa (NADH-coenzyme Q reductase) | CI-49 | 1q23.3 | |
| NDUFS3 | NADH dehydrogenase (ubiquinone) Fe-S protein 3, 30kDa (NADH-coenzyme Q reductase) | CI-30 | 11p11.11 | |
| NDUFS4 | NADH dehydrogenase (ubiquinone) Fe-S protein 4, 18kDa (NADH-coenzyme Q reductase) | AQDQ, CI-18 | 5q11.1 | |
| NDUFS5 | NADH dehydrogenase (ubiquinone) Fe-S protein 5, 15kDa (NADH-coenzyme Q reductase) | CI-15k | 1p34.2-p33 | |
| NDUFS6 | NADH dehydrogenase (ubiquinone) Fe-S protein 6, 13kDa (NADH-coenzyme Q reductase) | CI-13kA | 5p15.33 | |
| NDUFS7 | NADH dehydrogenase (ubiquinone) Fe-S protein 7, 20kDa (NADH-coenzyme Q reductase) | PSST, FLJ46880, FLJ45860, CI-20 | 19p13 | |
| NDUFS8 | NADH dehydrogenase (ubiquinone) Fe-S protein 8, 23kDa (NADH-coenzyme Q reductase) | TYKY, CI-23k | 11q13.2 | |
| NDUFV1 | NADH dehydrogenase (ubiquinone) flavoprotein 1, 51kDa | CI-51K | 11q13 | |
| NDUFV2 | NADH dehydrogenase (ubiquinone) flavoprotein 2, 24kDa | CI-24k | 18p11.22 | |
| NDUFV3 | NADH dehydrogenase (ubiquinone) flavoprotein 3, 10kDa | CI-10k | 21q22.3 |
2. Mitochondrial complex II: succinate dehydrogenase subunits
| Approved Symbol | Approved Name | Previous Symbols | Synonyms | Chromosome |
|---|---|---|---|---|
| SDHA | succinate dehydrogenase complex, subunit A, flavoprotein (Fp) | SDH2 | FP, SDHF | 5p15 |
| SDHB | succinate dehydrogenase complex, subunit B, iron sulfur (Ip) | SDH1, SDH | 1p36.1-p35 | |
| SDHC | succinate dehydrogenase complex, subunit C, integral membrane protein, 15kDa | PGL3 | 1q21 | |
| SDHD | succinate dehydrogenase complex, subunit D, integral membrane protein | PGL, PGL1 | 11q23 |
3. Mitochondrial complex III: ubiquinol-cytochrome c reductase complex subunits
| Approved Symbol | Approved Name | Previous Symbols | Synonyms | Chromosome |
|---|---|---|---|---|
| CYC1 | cytochrome c-1 | UQCR4 | 8q24 | |
| MT-CYB | mitochondrially encoded cytochrome b | MTCYB | COB, CYTB, UQCR3 | mitochondria |
| UQCR10 | ubiquinol-cytochrome c reductase, complex III subunit X | HSPC051, UCRC, QCR9, UCCR7.2 | 22q12.2 | |
| UQCR11 | ubiquinol-cytochrome c reductase, complex III subunit XI | UQCR | QCR10 | 19p13.3 |
| UQCRB | ubiquinol-cytochrome c reductase binding protein | UQBP | QP-C, QCR7, UQCR6 | 8q22 |
| UQCRC1 | ubiquinol-cytochrome c reductase core protein I | D3S3191, QCR1, UQCR1 | 3p21 | |
| UQCRC2 | ubiquinol-cytochrome c reductase core protein II | QCR2, UQCR2 | 16p12 | |
| UQCRFS1 | ubiquinol-cytochrome c reductase, Rieske iron-sulfur polypeptide 1 | RIS1, RIP1, UQCR5, RISP | 19q12 | |
| UQCRH | ubiquinol-cytochrome c reductase hinge protein | QCR6, UQCR8 | 1p34.1 | |
| UQCRQ | ubiquinol-cytochrome c reductase, complex III subunit VII, 9.5kDa | QP-C, QCR8, UQCR7 | 5q31.1 |
4. Mitochondrial complex IV: cytochrome c oxidase subunits
| Approved Symbol | Approved Name | Previous Symbols | Synonyms | Chromosome |
|---|---|---|---|---|
| COX4I1 | cytochrome c oxidase subunit IV isoform 1 | COX4 | COX4-1 | 16q24.1 |
| COX4I2 | cytochrome c oxidase subunit IV isoform 2 (lung) | COX4L2 | COXIV-2, COX4B, dJ857M17.2, COX4-2 | 20q11.21 |
| COX5A | cytochrome c oxidase subunit Va | 15q25 | ||
| COX5B | cytochrome c oxidase subunit Vb | 2q11.2 | ||
| COX6A1 | cytochrome c oxidase subunit VIa polypeptide 1 | COX6A | 12q24 | |
| COX6A2 | cytochrome c oxidase subunit VIa polypeptide 2 | 16p11.12 | ||
| COX6B1 | cytochrome c oxidase subunit VIb polypeptide 1 (ubiquitous) | COX6B | COXG | 19q13.1 |
| COX6B2 | cytochrome c oxidase subunit VIb polypeptide 2 (testis) | COXVIB2, FLJ32865, CT59 | 19q13.42 | |
| COX6C | cytochrome c oxidase subunit VIc | 8q22.2 | ||
| COX7A1 | cytochrome c oxidase subunit VIIa polypeptide 1 (muscle) | COX7A | COX7AH | 19q13.1 |
| COX7A2 | cytochrome c oxidase subunit VIIa polypeptide 2 (liver) | COXVIIa-L, COX7AL | 6q14.1 | |
| COX7B | cytochrome c oxidase subunit VIIb | Xq21.1 | ||
| COX7B2 | cytochrome c oxidase subunit VIIb2 | 4p12 | ||
| COX7C | cytochrome c oxidase subunit VIIc | 5q14 | ||
| COX8A | cytochrome c oxidase subunit VIIIA (ubiquitous) | COX8 | COX8-2, COX8L, VIII-L, COX, VIII | 11q12-q13 |
| COX8C | cytochrome c oxidase subunit VIIIC | COX8-3 | 14q32.13 | |
| MT-CO1 | mitochondrially encoded cytochrome c oxidase I | MTCO1 | COX1, COI | mitochondria |
| MT-CO2 | mitochondrially encoded cytochrome c oxidase II | MTCO2 | COX2, CO2 | mitochondria |
| MT-CO3 | mitochondrially encoded cytochrome c oxidase III | MTCO3 | COX3, COIII, CO3 | mitochondria |
5. Mitochondrial complex V: ATP synthase subunits
| Approved Symbol | Approved Name | Previous Symbols | Synonyms | Chromosome |
|---|---|---|---|---|
| ATP5A1 | ATP synthase, H+ transporting, mitochondrial F1 complex, alpha subunit 1, cardiac muscle | ATP5AL2, ATPM | ATP5A, hATP1, OMR, ORM | 18q21 |
| ATP5B | ATP synthase, H+ transporting, mitochondrial F1 complex, beta polypeptide | ATPSB | 12p13.3 | |
| ATP5C1 | ATP synthase, H+ transporting, mitochondrial F1 complex, gamma polypeptide 1 | ATP5CL1, ATP5C | 10p14 | |
| ATP5D | ATP synthase, H+ transporting, mitochondrial F1 complex, delta subunit | 19p13.3 | ||
| ATP5E | ATP synthase, H+ transporting, mitochondrial F1 complex, epsilon subunit | 20q13.3 | ||
| ATP5F1 | ATP synthase, H+ transporting, mitochondrial Fo complex, subunit B1 | 1p13.2 | ||
| ATP5G1 | ATP synthase, H+ transporting, mitochondrial Fo complex, subunit C1 (subunit 9) | ATP5G | 17q21.32 | |
| ATP5G2 | ATP synthase, H+ transporting, mitochondrial Fo complex, subunit C2 (subunit 9) | 12q13.13 | ||
| ATP5G3 | ATP synthase, H+ transporting, mitochondrial Fo complex, subunit C3 (subunit 9) | 2q31.1 | ||
| ATP5H | ATP synthase, H+ transporting, mitochondrial Fo complex, subunit d | ATPQ, ATP5JD | 17q25 | |
| ATP5I | ATP synthase, H+ transporting, mitochondrial Fo complex, subunit E | 4p16.3 | ||
| ATP5J | ATP synthase, H+ transporting, mitochondrial Fo complex, subunit F6 | ATP5A, ATP5, ATPM | CF6 | 21q21.1 |
| ATP5J2 | ATP synthase, H+ transporting, mitochondrial Fo complex, subunit F2 | F1Fo-ATPase, ATP5JL | 7q22.1 | |
| ATP5L | ATP synthase, H+ transporting, mitochondrial Fo complex, subunit G | ATP5JG | 11q23.3 | |
| ATP5L2 | ATP synthase, H+ transporting, mitochondrial Fo complex, subunit G2 | ATP5K2 | dJ222E13.5 | 22q13.33-qter |
| ATP5O | ATP synthase, H+ transporting, mitochondrial F1 complex, O subunit | OSCP, ATPO | 21q22 | |
| ATPIF1 | ATPase inhibitory factor 1 | ATPI, IP, ATPIP, MGC1167, MGC8898 | 1p35.3 | |
| MT-ATP6 | mitochondrially encoded ATP synthase 6 | MTATP6, RP | ATP6, ATPase-6, Su6m | mitochondria |
| MT-ATP8 | mitochondrially encoded ATP synthase 8 | MTATP8 | ATP8, A6L | mitochondria |
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