Recombinant Human SCO2 protein, His-tagged

• Cat.No.: SCO2-3449H
• Product Overview: Recombinant Human SCO2(42-266aa) fused with His tag at N-terminal was expressed in E. coli.

Specification

• Species: Human
• Source: E.coli
• Tag: His
• Protein Length: 42-266 a.a.
• 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.
• Form: Liquid. 20mM Tris-HCl buffer (pH8.0) containing 30% glycerol, 2mM DTT, 200mM NaCl
• Molecular Mass: 27.4kDa (246aa), confirmed by MALDI-TOF
• AA Sequence: MGSSHHHHHH SSGLVPRGSH MGRARCKADFR GQWVLMYFGF THCPDICPDE LEKLVQVVRQ LEAEPGLPPV QPVFITVDPE RDDVEAMARY VQDFHPRLLG LTGSTKQVAQ GPAETGGQG QPQGPGLRTR LLITGLFGAG LGGAWLALRA EKERLQQQKR TEALRQAAVG QGDFHLLDHRASHSYRVYYN AGPKDEDQDY IVDHSIAIYL LNPDGLFTDY YGRSRSAEQI SDSVRRHMAA FRSVLS
• Purity: >90% by SDS - PAGE
• Storage: Can be stored at +4 centigrade short term (1-2 weeks). For long term storage, aliquot and store at -20 centigrade or -70 centigrade. Avoid repeated freezing and thawing cycles.
• Concentration: 0.5 mg/ml (determined by Bradford assay)

Gene Information

• Gene Name: SCO2 SCO cytochrome oxidase deficient homolog 2 (yeast) [ Homo sapiens ]
• Official Symbol: SCO2
• Synonyms: SCO2; SCO cytochrome oxidase deficient homolog 2 (yeast); SCO (cytochrome oxidase deficient, yeast) homolog 2; protein SCO2 homolog, mitochondrial; SCO1L; cytochrome oxidase deficient homolog 2; MGC125823; MGC125825
• Gene ID: 9997
• mRNA Refseq: NM_005138
• Protein Refseq: NP_005129
• MIM: 604272
• UniProt ID: O43819
• Chromosome Location: 22q13.33
• Pathway: salvage pathways of pyrimidine deoxyribonucleotides, organism-specific biosystem
• Function: copper ion binding; metal ion binding