Cytochrome C Oxidase Subunit I (COX1)

MT-CO1; MTCO1; COI; COXI; Mitochondrially Encoded Cytochrome C Oxidase I; Cytochrome c oxidase polypeptide I

SPN	T498
HGNC	7419
MGI	102504
PubMed	23362268
RGD	621871
UniProt	P00395
Wiki	MT-CO1

MT-CO1 belongs to cytochrome c oxidase subunit I protein family. It is often used in DNA barcoding, because its mutation rate is often fast enough to distinguish closely related species. In most if not all seed plants, however, the rate of evolution of cox1 is very slow.

The enzyme complex consists of 3-4 subunits (prokaryotes) up to 13 polypeptides (mammals) of which only the catalytic subunit (equivalent to mammalian subunit I (CO I)) is found in all heme-copper respiratory oxidases. The presence of a bimetallic centre (formed by a high-spin heme and copper B) as well as a low-spin heme, both ligated to six conserved histidine residues near the outer side of four transmembrane spans within CO I is common to all family members.

Reference

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"Introducing a novel human mtDNA mutation into the Paracoccus denitrificans COX I gene explains functional deficits in a patient."
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"A mitochondrial DNA mutation linked to colon cancer results in proton leaks in cytochrome c oxidase."
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