COX2 rabbit pAb - 50 μL

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 multisystem disease, with onset from infancy to adulthood. 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.