Recombinant Human SCO Cytochrome Oxidase Deficient Homolog 2

Source : Escherichia Coli. SCO2 produced in E.Coli is a single, non-glycosylated polypeptide chain containing 246 amino acids (42-266a.a.) and having a molecular mass of 27.4kDa.SCO2 is fused to a 21 amino acid His-tag at N-terminus & purified by proprietary chromatographic techniques. SCO2 protein is a member of the SCO1/2 family. SCO1 and SCO2 proteins are found on the inner membrane of the mitochondria and takes a vital part copper insertion or transport to the active site of cytochrome c oxidase (COX) . Flaws in SCO2 are the reason for deadly infantile cardioencephalomyopathy with cytochrome c oxidase deficiency (FIC) which is characterized by hypertrophic cardiomyopathy, lactic acidosis, and gliosis. Heart and skeletal muscle display declines in cytochrome c oxidase (COX) activity, while liver and fibroblasts show mild COX deficiencies.