Recombinant human SCO2 protein
€0.00
In stock
SKU
ATGP0867
Description
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.Alternative Names
Protein SCO2 homolog mitochondrial, Protein SCO2 homolog mitochondrial, SCO1LConcentration
0.5mg/ml (determined by Bradford assay)Concentration
Liquid in. 20mM Tris-HCl buffer (pH 8.0) containing 30% glycerol, 2mM DTT, 200mM NaClStorage: Can be stored at +2C to +8C for 1 week. For long term storage, aliquot and store at -20C to -80C. Avoid repeated freezing and thawing cycles.
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.Alternative Names
Protein SCO2 homolog mitochondrial, Protein SCO2 homolog mitochondrial, SCO1LConcentration
0.5mg/ml (determined by Bradford assay)Concentration
Liquid in. 20mM Tris-HCl buffer (pH 8.0) containing 30% glycerol, 2mM DTT, 200mM NaClStorage: Can be stored at +2C to +8C for 1 week. For long term storage, aliquot and store at -20C to -80C. Avoid repeated freezing and thawing cycles.
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