Cytochrome c oxidase (COX), the terminal enzyme of the mitochondrial respiratory chain, catalyzes the electron transfer from reduced cytochrome c to oxygen. It is a heteromeric complex consisting of 3 catalytic subunits encoded by mitochondrial genes and multiple structural subunits encoded by nuclear genes. The mitochondrially-encoded subunits function in the electron transfer and the nuclear-encoded subunits may function in the regulation and assembly of the complex. This nuclear gene encodes polypeptide 1 (liver isoform) of subunit VIa, and polypeptide 1 is found in all non-muscle tissues. Polypeptide 2 (heart/muscle isoform) of subunit VIa is encoded by a different gene, and is present only in striated muscles. These two polypeptides share 66% amino acid sequence identity. It has been reported that there may be several pseudogenes on chromosomes 1, 6, 7q21, 7q31-32 and 12. However, only one pseudogene (COX6A1P) on chromosome 1p31.1 has been documented.
COX6A1; cytochrome c oxidase subunit VIa polypeptide 1; COX6A; cytochrome c oxidase subunit 6A1, mitochondrial; mitochondrial; COX VIa L; COX VIa-L; COX6AL; CX6A1_HUMAN; Cytochrome c oxidase polypeptide VIa liver; Cytochrome c oxidase polypeptide VIa-liver; Cytochrome c oxidase subunit 6A1; Cytochrome c oxidase subunit 6A1 mitochondrial; Cytochrome C oxidase subunit VIa homolog; Cytochrome c oxidase subunit VIA liver; Cytochrome c oxidase subunit VIA-liver; MGC104500; MS412; ns:zf-e250; zgc:103782