Cytochrome c oxidase subunit 8A (COX8A)

Cytochrome c oxidase subunit 8A (COX8A) is a nuclear-encoded protein component of cytochrome c oxidase, also known as complex IV, the terminal enzyme of the mitochondrial respiratory chain[1][2][3][7][9][12][15]. COX8A forms part of the multi-subunit oxidoreductase complex responsible for transferring electrons from cytochrome c to molecular oxygen, thus driving ATP synthesis via oxidative phosphorylation. The subunit is crucial for proper assembly and/or regulation of the enzyme complex but does not directly participate in catalysis. Mutations in COX8A disrupt mitochondrial respiration, leading to mitochondrial complex IV deficiency, presenting clinically as Leigh syndrome, epileptic encephalopathy, and other severe neurological disorders. There are currently no specific drugs or targeted therapies for COX8A itself; rather, the wider complex IV is sensitive to inhibitors such as cyanide, but these are not therapeutic agents[1][2][7][8][10].

Other names

COX8COX8LCOX8-2VIII-LCOXVIIIcytochrome c oxidase polypeptide VIII-liver/heartcytochrome c oxidase subunit 8-2cytochrome c oxidase subunit VIIIAMC4DN15

Not targeted directly by therapeutics; drugs interacting with cytochrome c oxidase generally inhibit complex IV function, blocking electron transfer and oxidative phosphorylation (e.g., cyanide, carbon monoxide) but no clinical therapeutics target COX8A itself