Cytochrome c oxidase subunit III

Cytochrome c oxidase subunit III (COX3) is an enzyme that in humans is encoded by the MT-CO3 gene. It is one of main transmembrane subunits of cytochrome c oxidase. It is also one of the three mitochondrial DNA (mtDNA) encoded subunits (MT-CO1, MT-CO2, MT-CO3) of respiratory complex IV. Variants of it have been associated with isolated myopathy, severe encephalomyopathy, Leber hereditary optic neuropathy, mitochondrial complex IV deficiency, and recurrent myoglobinuria .

COX3
Identifiers
AliasesCOX3, COIII, MTCO3, Cytochrome c oxidase subunit III, cytochrome c oxidase III
External IDsOMIM: 516050 MGI: 102502 HomoloGene: 5014 GeneCards: COX3
Orthologs
SpeciesHumanMouse
Entrez

4514

17710

Ensembl

ENSG00000198938

ENSMUSG00000064358

UniProt

P00414

P00416

RefSeq (mRNA)

n/a

n/a

RefSeq (protein)

n/a

NP_904334

Location (UCSC)Chr M: 0.01 – 0.01 MbChr M: 0.01 – 0.01 Mb
PubMed search
Wikidata
View/Edit HumanView/Edit Mouse
Cytochrome c oxidase subunit III
Structure of the 13-subunit oxidized cytochrome c oxidase.
Identifiers
SymbolCOX3
PfamPF00510
InterProIPR000298
PROSITEPDOC50253
SCOP21occ / SCOPe / SUPFAM
TCDB3.D.4
OPM superfamily4
OPM protein1v55
CDDcd01665
Available protein structures:
Pfam  structures / ECOD  
PDBRCSB PDB; PDBe; PDBj
PDBsumstructure summary
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