COX3 is a multi-pass membrane protein. It belongs to the cytochrome c oxidase subunit 3 family. Defects in COX3 are a cause of Leber hereditary optic neuropathy (LHON) and cytochrome c oxidase deficiency (COX deficiency). Defects in MT-CO3 are also found in mitochondrial encephalomyopathy with lactic acidosis and stroke-like episodes (MELAS) syndrome and recurrent myoglobinuria.
COIII; CytoChrome c oxidase III; Cytochrome c oxidase polypeptide III; Cytochrome c oxidase subunit 3; MTCO3