Mitochondrial respiratory chain complex
Useful external resources:
- Mitochondrial complex I homepage Scripps Research Institute
- Mitochondrial complex II homepage Berkeley University
- Mitochondrial complex III - Cytochrome bc1 project Berkeley University
- Mitochondrial complex IV - General introduction University of Illinois at Urbana-Champaign
- Mitochondrial complex IV homepage Rice University, Texas
- Mitochondrial complex V - ATP synthase web page Boris A. Feniouk
You can view genes encoding assembly factors for these complexes at our Mitochondrial respiratory chain complex assembly factors page.
Click on the Approved Gene Symbol to retrieve the complete gene record
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 | 22q13.2 |
| 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 |
This online database does not include our confidential data. If you would like to create a new gene symbol and name, the only way to confirm it as unique is by contacting us.
