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|Product Name||SCO2, 42-266aa, Human|
|Amino Acid Sequence||MGSSHHHHHH SSGLVPRGSH MGPAETGGQG QPQGPGLRTR LLITGLFGAG LGGAWLALRA EKERLQQQKR TEALRQAAVG QGDFHLLDHR GRARCKADFR GQWVLMYFGF THCPDICPDE LEKLVQVVRQ LEAEPGLPPV QPVFITVDPE RDDVEAMARY VQDFHPRLLG LTGSTKQVAQ ASHSYRVYYN AGPKDEDQDY IVDHSIAIYL LNPDGLFTDY YGRSRSAEQI SDSVRRHMAA FRSVLS|
|Background||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.|
|Concentration||1.0 mg/ml (determined by Bradford assay)|
|Form||Liquid, in 20mM Tris-HCl buffer (pH8.0) containing 20% glycerol, 0.1M NaCl, 1mM DTT|
|Intended Use||For Research Use Only|
|Molecular Weight||21.6 kDa (190aa), confirmed by MALDI-TOF|
|NCBI Accession #||NP_005129|
|Purity||> 90% by SDS-PAGE|
|References||Meister G., et al. (2001) EMBO J. 20:2304-2314
Talbot K., et al. (1998) Hum. Mol. Genet. 7:2149-2156
|Storage||Can be stored at +4C short term (1-2 weeks). For long term storage, aliquot and store at -20C or -70C. Avoid repeated freezing and thawing cycles.|
|Synonyms||Protein SCO2 homolog mitochondrial, SCO1L|
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