SCO2 protein (His tag) (80R-1626)
Purified recombinant Human SCO2 protein
|Synonyms||SCO2, SCO-2 protein, Protein SCO2 homolog mitochondrial protein, SCO-2, SCO 2 protein, SCO 2, SCO1L|
Coomassie Blue stained SDS-PAGE of SCO2 protein (His tag) (80R-1626)
Figure annotation denotes ug of protein loaded and % gel used.
|Residues||42-266 amino acids: 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|
|Grade & Purity||> 90% pure|
|Molecular Weight||27.4 kDa (246aa), confirmed by MALDI-TOF|
|Form & Buffer||Supplied as a liquid in 20mM Tris-HCl buffer, pH 8.0, containing 30% glycerol, 2mM DTT and 200mM NaCl.|
Storage & Safety
|Storage||Store at 4 deg C for short term storage (1/2 weeks). Aliquot and store at -20 deg C or - 70 deg C for long term storage. Avoid repeated freeze/thaw cycles.|
|Biological Significance||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.|
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