|Other Names||Protein SCO2 homolog, mitochondrial, SCO2|
|Format||Synthetic peptide was lyophilized with 100% acetonitrile and is supplied as a powder. Reconstitute with 0.1 ml DI water for a final concentration of 1 mg/ml.|
|Storage||Maintain refrigerated at 2-8°C for up to 6 months. For long term storage store at -20°C.|
|Precautions||This product is for research use only. Not for use in diagnostic or therapeutic procedures.|
|Function||Copper metallochaperone essential for the synthesis and maturation of cytochrome c oxidase subunit II (MT-CO2/COX2). Involved in transporting copper to the Cu(A) site on MT-CO2/COX2 (PubMed:15229189, PubMed:17189203). Also acts as a thiol-disulfide oxidoreductase to regulate the redox state of the cysteines in SCO1 during maturation of MT-CO2/COX2 (PubMed:19336478).|
|Cellular Location||Mitochondrion inner membrane; Single-pass membrane protein|
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Provided below are standard protocols that you may find useful for product applications.
Cytochrome c oxidase (COX) catalyzes the transfer ofelectrons from cytochrome c to molecular oxygen, which helps tomaintain the proton gradient across the inner mitochondrialmembrane that is necessary for aerobic ATP production. Human COX isa multimeric protein complex that requires several assemblyfactors; this gene encodes one of the COX asembly factors. Theencoded protein is a metallochaperone that is involved in thebiogenesis of cytochrome c oxidase subunit II. Mutations in thisgene are associated with fatal infantile encephalocardiomyopathy.
Bailey, S.D., et al. Diabetes Care 33(10):2250-2253(2010)Foltopoulou, P.F., et al. Biochim. Biophys. Acta 1802(6):497-508(2010)Pronicki, M., et al. Eur. J. Paediatr. Neurol. 14(3):253-260(2010)Kamatani, Y., et al. Nat. Genet. 42(3):210-215(2010)Talmud, P.J., et al. Am. J. Hum. Genet. 85(5):628-642(2009)
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