Recombinant Human SCO Cytochrome Oxidase Deficient Homolog 2
供应商
ProSpec
产品货号
PRO-054
产品报价
¥询价/10ug ¥1mg ¥2ug
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产品新闻
背景资料
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.