Anti-CBS Antibody, clone 9F3.2

产品说明

一般描述

Cystathionine beta-synthase (EC 4.2.1.22; UniProt P35520; also known as beta-thionase, Methylcysteine synthase, Serine sulfhydrase) is encoded by the CBS (also known as HIP4) gene (Gene ID 875) in human. Cystathionine beta-synthase catalyzes the trans-sulfuration of homocysteine and serine to form the cysteine precursor cystathionine in the folate pathway. Cysteine is an essential substrate for the biosynthesis of glutathione, a major endogenous antioxidant. CBS deficiency results in altered sulfur metabolism and homocystinuria characterized by increased urinary homocystine and methionine. The clinical features of untreated homocystinuria due to CBS deficiency include myopia, ectopia lentis, mental retardation, skeletal anomalies resembling Marfan syndrome, and thromboembolic events.

特异性

Expected to react with both the Major (isoform 1) and the Minor (isoform 2) spliced variant.

免疫原

Recombinant protein corresponding to the N-terminal of human CBS.

应用

Immunohistochemistry Analysis: A 1:1,000 dilution from a representative lot detected CBS in human liver and human pancreas tissue.
Anti-CBS Antibody, clone 9F3.2 is an antibody against CBS for use in Western Blotting, Immunohistochemistry (Paraffin).

质量

Evaluated by Western Blotting in Raji cell lysate.

Western Blotting Analysis: 0.5 µg/mL of this antibody detected CBS in 10 µg of Raji cell lysate.

目标描述

~61 kDa observed

外形

Protein G Purified
Format: Purified

其他说明

Concentration: Please refer to lot specific datasheet.

免责声明

Unless otherwise stated in our catalog or other company documentation accompanying the product(s), our products are intended for research use only and are not to be used for any other purpose, which includes but is not limited to, unauthorized commercial uses, in vitro diagnostic uses, ex vivo or in vivo therapeutic uses or any type of consumption or application to humans or animals.

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