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Mitochondrial respiratory chain complex

 

1. Mitochondrial complex I: NADH dehydrogenase (ubiquinone) subunits
2. Mitochondrial complex II: succinate dehydrogenase subunits
3. Mitochondrial complex III: ubiquinol-cytochrome c reductase complex subunits
4. Mitochondrial complex IV: cytochrome c oxidase subunits
5. Mitochondrial complex V: ATP synthase subunits

Useful external resources:

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:ubiquinone oxidoreductase core subunit 1 MTND1 ND1, NAD1 mitochondria
MT-ND2 mitochondrially encoded NADH:ubiquinone oxidoreductase core subunit 2 MTND2 ND2, NAD2 mitochondria
MT-ND3 mitochondrially encoded NADH:ubiquinone oxidoreductase core subunit 3 MTND3 ND3, NAD3 mitochondria
MT-ND4 mitochondrially encoded NADH:ubiquinone oxidoreductase core subunit 4 MTND4, LHON ND4, NAD4 mitochondria
MT-ND4L mitochondrially encoded NADH:ubiquinone oxidoreductase core subunit 4L MTND4L ND4L, NAD4L mitochondria
MT-ND5 mitochondrially encoded NADH:ubiquinone oxidoreductase core subunit 5 MTND5 ND5, NAD5 mitochondria
MT-ND6 mitochondrially encoded NADH:ubiquinone oxidoreductase core subunit 6 MTND6 NAD6, ND6 mitochondria
NDUFA1 NADH:ubiquinone oxidoreductase subunit A1 MWFE, CI-MWFE Xq24
NDUFA2 NADH:ubiquinone oxidoreductase subunit A2 B8 5q31.2
NDUFA3 NADH:ubiquinone oxidoreductase subunit A3 B9 19q13.42
NDUFA5 NADH:ubiquinone oxidoreductase subunit A5 B13, NUFM, CI-13KD-B, UQOR13, CI-13kB 7q31.33
NDUFA6 NADH:ubiquinone oxidoreductase subunit A6 B14, LYRM6, CI-B14, NADHB14 22q13.2
NDUFA7 NADH:ubiquinone oxidoreductase subunit A7 B14.5a 19p13.2
NDUFA8 NADH:ubiquinone oxidoreductase subunit A8 PGIV, MGC793 9q33.2
NDUFA9 NADH:ubiquinone oxidoreductase subunit A9 NDUFS2L SDR22E1, CI-39k 12p13.3
NDUFA10 NADH:ubiquinone oxidoreductase subunit A10 CI-42k 2q37.3
NDUFA11 NADH:ubiquinone oxidoreductase subunit A11 B14.7 19p13.3
NDUFA12 NADH:ubiquinone oxidoreductase subunit A12 DAP13, B17.2 12q22
NDUFA13 NADH:ubiquinone oxidoreductase subunit A13 CGI-39, CDA016, GRIM-19, GRIM19, B16.6 19p13.11
NDUFAB1 NADH:ubiquinone oxidoreductase subunit AB1 SDAP, FASN2A, ACP 16p12.3
NDUFB1 NADH:ubiquinone oxidoreductase subunit B1 MNLL, CI-MNLL 14q31.3
NDUFB2 NADH:ubiquinone oxidoreductase subunit B2 AGGG, CI-AGGG 7q34
NDUFB3 NADH:ubiquinone oxidoreductase subunit B3 B12 2q33.1
NDUFB4 NADH:ubiquinone oxidoreductase subunit B4 B15 3q13.33
NDUFB5 NADH:ubiquinone oxidoreductase subunit B5 SGDH, CI-SGDH, MGC12314 3q27.1
NDUFB6 NADH:ubiquinone oxidoreductase subunit B6 B17, CI 9p13.2
NDUFB7 NADH:ubiquinone oxidoreductase subunit B7 B18, CI-B18, MGC2480 19p13.12
NDUFB8 NADH:ubiquinone oxidoreductase subunit B8 ASHI, CI-ASHI 10q24.31
NDUFB9 NADH:ubiquinone oxidoreductase subunit B9 B22, UQOR22, LYRM3 8q24.13
NDUFB10 NADH:ubiquinone oxidoreductase subunit B10 PDSW 16p13.3
NDUFB11 NADH:ubiquinone oxidoreductase subunit B11 ESSS, NP17.3, Np15 Xp11.3
NDUFC1 NADH:ubiquinone oxidoreductase subunit C1 KFYI 4q31.1
NDUFC2 NADH:ubiquinone oxidoreductase subunit C2 B14.5b, HLC-1 11q14.1
NDUFS1 NADH:ubiquinone oxidoreductase core subunit S1 CI-75k 2q33-q34
NDUFS2 NADH:ubiquinone oxidoreductase core subunit S2 CI-49 1q23.3
NDUFS3 NADH:ubiquinone oxidoreductase core subunit S3 CI-30 11p11.11
NDUFS4 NADH:ubiquinone oxidoreductase subunit S4 AQDQ, CI-18 5q11.1
NDUFS5 NADH:ubiquinone oxidoreductase subunit S5 CI-15k 1p34.2-p33
NDUFS6 NADH:ubiquinone oxidoreductase subunit S6 CI-13kA 5p15.33
NDUFS7 NADH:ubiquinone oxidoreductase core subunit S7 PSST, FLJ46880, FLJ45860, CI-20 19p13
NDUFS8 NADH:ubiquinone oxidoreductase core subunit S8 TYKY, CI-23k 11q13.2
NDUFV1 NADH:ubiquinone oxidoreductase core subunit V1 CI-51K 11q13
NDUFV2 NADH:ubiquinone oxidoreductase core subunit V2 CI-24k 18p11.22
NDUFV3 NADH:ubiquinone oxidoreductase subunit V3 CI-10k 21q22.3

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2. Mitochondrial complex II: succinate dehydrogenase subunits

Approved Symbol Approved Name Previous Symbols Synonyms Chromosome
SDHA succinate dehydrogenase complex flavoprotein subunit A SDH2 FP, SDHF 5p15
SDHB succinate dehydrogenase complex iron sulfur subunit B SDH1, SDH 1p36.1-p35
SDHC succinate dehydrogenase complex subunit C PGL3 CYB560, cybL 1q23.3
SDHD succinate dehydrogenase complex subunit D PGL, PGL1 cybS 11q23

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3. Mitochondrial complex III: ubiquinol-cytochrome c reductase complex subunits

Approved Symbol Approved Name Previous Symbols Synonyms Chromosome
CYC1 cytochrome c1 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 QP-C, QCR8, UQCR7 5q31.1

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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

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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

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