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Alternative Name : |
SCO1L, SCO Cytochrome Oxidase Deficient Homolog 2 (yeast), Protein SCO2 Homolog-Mitochondrial, MGC125823, MGC125825. |
Amount : |
10 µg |
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.