(SCO2 Cytochrome C Oxidase Assembly Protein (SCO2))
Cytochrome c oxidase (COX) catalyzes the transfer of electrons from cytochrome c to molecular oxygen, which helps to maintain the proton gradient across the inner mitochondrial membrane that is necessary for aerobic ATP production. Human COX is a multimeric protein complex that requires several assembly factors\; this gene encodes one of the COX asembly factors. The encoded protein is a metallochaperone that is involved in the biogenesis of cytochrome c oxidase subunit II. Mutations in this gene are associated with fatal infantile encephalocardiomyopathy. [provided by RefSeq, Dec 2009].
Soma, Morgada, Naik, Boulet, Roesler, Dziuba, Ghosh, Yu, Lindahl, Ames, Leary, Vila, Gohil: "COA6 Is Structurally Tuned to Function as a Thiol-Disulfide Oxidoreductase in Copper Delivery to Mitochondrial Cytochrome c Oxidase." in: Cell reports, Vol. 29, Issue 12, pp. 4114-4126.e5, (2020) (PubMed).
Matoba, Kang, Patino, Wragg, Boehm, Gavrilova, Hurley, Bunz, Hwang: "p53 regulates mitochondrial respiration." in: Science (New York, N.Y.), Vol. 312, Issue 5780, pp. 1650-3, (2006) (PubMed).
Horng, Leary, Cobine, Young, George, Shoubridge, Winge: "Human Sco1 and Sco2 function as copper-binding proteins." in: The Journal of biological chemistry, Vol. 280, Issue 40, pp. 34113-22, (2005) (PubMed).
Papadopoulou, Sue, Davidson, Tanji, Nishino, Sadlock, Krishna, Walker, Selby, Glerum, Coster, Lyon, Scalais, Lebel, Kaplan, Shanske, De Vivo, Bonilla, Hirano, DiMauro, Schon: "Fatal infantile cardioencephalomyopathy with COX deficiency and mutations in SCO2, a COX assembly gene." in: Nature genetics, Vol. 23, Issue 3, pp. 333-7, (1999) (PubMed).
Glerum, Shtanko, Tzagoloff: "SCO1 and SCO2 act as high copy suppressors of a mitochondrial copper recruitment defect in Saccharomyces cerevisiae." in: The Journal of biological chemistry, Vol. 271, Issue 34, pp. 20531-5, (1996) (PubMed).
Aliases for SCO2 抗体
SCO cytochrome oxidase deficient homolog 2 (yeast) (SCO2) 抗体 SCO2 cytochrome c oxidase assembly protein (Sco2) 抗体 SCO2, cytochrome c oxidase assembly protein (SCO2) 抗体 CEMCOX1 抗体 MYP6 抗体 SCO1L 抗体