Recombinant human SCO2 protein
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.
Product Specifications
Product Name Alternative
Protein SCO2 homolog mitochondrial, Protein SCO2 homolog mitochondrial, SCO1L
Expression System
E.coli
Antigen Species
Human
Tag
His-Tag
Applications
SDS-PAGE
Concentration
0.5 mg/mL (determined by Bradford assay)
Purity
> 90% by SDS-PAGE
Molecular Weight
27.4 kDa (246aa) confirmed by MALDI-TOF
Additionnal Information
References & Citations
Meister G., et al. (2001) EMBO J. 20:2304-2314; ; Talbot K., et al. (1998) Hum. Mol. Genet. 7:2149-2156;
Storage Conditions
Can be stored at 2°C to 8°C for 1 week. For long term storage, aliquot and store at -20C to -80C. Avoid repeated freezing and thawing cycles.
Formulation
Liquid in. 20 mM Tris-HCl buffer (pH 8.0) containing 30% glycerol, 2mM DTT, 200 mM NaCl
Scientific Category
Metabolism
NCBI Accession Number
NP_005129
Uniprot Accession Number
O43819
Species
Human
AA Sequence
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