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SCO2 Polyclonal Antibody
SCO2 Polyclonal Antibody
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SCO2 Polyclonal Antibody
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经销商客户: ¥214.5
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商品描述

商品属性

Target:SCO2

Fields:Central carbon metabolism in cancer

Gene Name:SCO2

Protein Name:Protein SCO2 homolog, mitochondrial

Human Gene Id:9997

Human Swiss Prot No:O43819

Mouse Swiss Prot No:Q8VCL2

Immunogen:Synthesized peptide derived from human protein . at AA range: 181-230

Specificity:SCO2 Polyclonal Antibody detects endogenous levels of protein.

Formulation:Liquid in PBS containing 50% glycerol, and 0.02% sodium azide.

Source:Polyclonal, Rabbit,IgG

Dilution:WB 1:500-2000 ELISA 1:5000-20000

Purification:The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.

Concentration:1 mg/ml

Storage Stability:-15°C to -25°C/1 year(Do not lower than -25°C)

Observed Band(KD):29kD

Background: 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],

Function: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.,

Subcellular Location:Mitochondrion inner membrane ; Single-pass membrane protein .

Expression:Ubiquitous.

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