SCO1 polyclonal antibody

The SCO1 and SCO2 protein homologs belong to the SCO1/2 family of proteins. SCO1 and SCO2 both localize to the mitochondrion and are inner membrane proteins crucial for copper insertion or transport to the active site of cytochrome c oxidase (COX). COX is a crucial component in energy production because it functions as the terminal enzyme in the respiratory chain. SCO1 is predominantly expressed in highly oxidative phosphorylation tissues such as brain, heart and muscle, while SCO2 is ubiquitously expressed. Defects in the gene encoding for SCO1 may cause cytochrome c oxidase deficiency, a hetero-genous disorder. Defects in the gene encoding for SCO2 may cause cardioencephalomyopathy with cytochrome c oxidase deficiency, a fatal infantile disorder characterized by hypertrophic cardiomyopathy, lactic acidosis and gliosis.