Cell localization:Cytoplasmic.cofactor:Copper A.,disease:Defects in MT-CO2 are a cause of cytochrome c oxidase deficiency (COX deficiency) [MIM:220110], also called mitochondrial complex IV deficiency. COX deficiency is a clinically heterogeneous disorder. The clinical features are ranging from isolated myopathy to severe mµLtisystem disease, with onset from infancy to adµLthood.,disease:Defects in MT-CO2 are associated with tumor formation.,function:Cytochrome c oxidase is the component of the respiratory chain that catalyzes the reduction of oxygen to water. Subunits 1-3 form the functional core of the enzyme complex. Subunit 2 transfers the electrons from cytochrome c via its binuclear copper A center to the bimetallic center of the catalytic subunit 1.,similarity:Belongs to the cytochrome c oxidase subunit 2 family.,
