Recombinant Human SCO Cytochrome Oxidase Deficient Homolog 2

Recombinant Human SCO Cytochrome Oxidase Deficient Homolog 2

Catalog Number:
P001414016ABE
Mfr. No.:
Abe32-4780
Price:
  • Size:
    10 µg
    Quantity:
    Add to Cart:
      • Overview
        • 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.

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      • Properties
        • Protein Name
          SCO1L, SCO Cytochrome Oxidase Deficient Homolog 2 (yeast), Protein SCO2 Homolog-Mitochondrial, MGC125823, MGC125825.
          Source
          E. coli
          Type
          Recombinant Proteins
          Purification
          Greater than 90% as determined by SDS-PAGE.
          Formulation
          The SCO2 protein solution (0.5mg/1ml) is formulated in 20mM Tris-HCl buffer (pH8.0) 2mM DTT, 200mM NaCl and 30% glycerol.
          Storage
          Store at 4°C if entire vial will be used within 2-4 weeks. Store, frozen at -20°C for longer periods of time. Please avoid freeze thaw cycles.

          * For Research Use Only. Not for use in diagnostic/therapeutics procedures.

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