Product Description

SCO2 Rabbit Polyclonal Antibody

Sizes: 50μL, 100μL

Catalogue Numbers: ES11415-50, ES11415-100

Lead times: 1-2 weeks, if manufacturer has product in stock

Manufacturer/Ship Location: China

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],

Reactivity: Human, Mouse

Host: Rabbit

Applications: WB, ELISA

Clonality: Polyclonal

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

Isotype: IgG

Target: SCO2

Specificity: SCO2 Polyclonal Antibody detects endogenous levels of protein.

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,

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

Expression: Ubiquitous,

Observed Band (KD): 29kD

Fields: Central carbon metabolism in cancer

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

Storage and Stability: -20°C/1 year

Gene Name: SCO2

Protein Name: Protein SCO2 homolog, mitochondrial

Mouse SwissProt Number: Q8VCL2

Research Use Only