Purity: > 90% by SDS - PAGE Format: Liquid. 20mM Tris-HCl buffer (pH8.0) containing 30% glycerol, 2mM DTT, 200mM NaCl . SCO2 protein belongs to the SCO1/2 family of proteins. Both SCO1 and SCO2 proteins are located on the inner membrane of the mitochondria and plays a crucial role in copper insertion or transport to the active site of cytochrome c oxidase (COX). Defects in SCO2 are the cause of fatal infantile cardioencephalomyopathy with cytochrome c oxidase deficiency (FIC). This disease is characterized by hypertrophic cardiomyopathy, lactic acidosis, and gliosis. Heart and skeletal muscle show reductions in cytochrome c oxidase (COX) activity, whereas liver and fibroblasts show mild COX deficiencies. Recombinant human SCO2 protein, fused to His-tag at N-terminus, was expressed in E.coli and purified by using conventional chromatography techniques.
Size
250ug, 50ug, 10ug
Concentration
n/a
Applications
SDS-PAGE
Other Names
Protein SCO2 homolog mitochondrial, Protein SCO2 homolog mitochondrial, SCO1L