产品名称
SCO2 Rabbit Polyclonal Antibody
蛋白名称
Protein SCO2 homolog, mitochondrial
存储缓冲液
Liquid in PBS containing 50% glycerol, and 0.02% New type preservative N.
Human Swissprot No.
O43819
Human Swissprot Link
https://www.uniprot.org/uniprotkb/O43819/entry
Mouse Swissprot No.
Q8VCL2
Mouse Swissprot Link
http://www.uniprot.org/uniprot/Q8VCL2
免疫原
Synthesized peptide derived from human protein . at AA range: 181-230
特异性
SCO2 Polyclonal Antibody detects endogenous levels of protein.
稀释度
WB 1:500-2000 ELISA 1:5000-20000
宿主
Polyclonal, Rabbit,IgG
背景介绍
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 assembly 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 and myopia 6. [provided by RefSeq, Oct 2014],
细胞定位
Mitochondrion inner membrane ; Single-pass membrane protein .
功能
disease:Defects in SCO2 are the cause of fatal infantile cardioencephalomyopathy with cytochrome c oxidase deficiency (FIC) [MIM:604377, 220110]. 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.,function:Acts as a copper chaperone, transporting copper to the Cu(A) site on the cytochrome c oxidase subunit II (COX2).,similarity:Belongs to the SCO1/2 family.,similarity:Contains 1 thioredoxin domain.,tissue specificity:Ubiquitous.,
纯化
The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.
