Background of the antigen
Cytochrome C Oxidase subunits I, II and III form the functional core of the enzyme complex.Defects in Cytochrome C Oxidase subunits III are a cause of Leber hereditary optic neuropathy (LHON) and cytochrome c oxidase deficiency (COX deficiency). Defects in Cytochrome C Oxidase subunits III are also found in mitochondrial encephalomyopathy with lactic acidosis and stroke-like episodes (MELAS) syndrome and recurrent myoglobinuria.