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重组人细胞色素氧化酶缺陷同源物2/SCO2 Human

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重组人细胞色素氧化酶缺陷同源物2/SCO2 Human
产品编号:PRO-054产品名称:重组人细胞色素氧化酶缺陷同源物2/SCO2 Human产品规格:2ug/10ug/1mg
Catalogue NumberPRO-054
Synonyms SCO1L, SCO Cytochrome Oxidase Deficient Homolog 2 (yeast), Protein SCO2 Homolog-Mitochondrial, MGC125823, MGC125825.
Introduction 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.
Description 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.
Source Escherichia Coli.
Physical Appearance Sterile Filtered clear solution.
Formulation The SCO2 protein solution (0.5mg/1ml) is formulated in 20mM Tris-HCl buffer (pH8.0) 2mM DTT, 200mM NaCl and 30% glycerol.
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
Purity Greater than 90% as determined by SDS-PAGE.
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.
Usage ProSpecs products are furnished for LABORATORY RESEARCH USE ONLY. The product may not be used as drugs, agricultural or pesticidal products, food additives or household chemicals.

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