Application

Anti-CBS Antibody, clone 9F3.2 is an antibody against CBS for use in Western Blotting, Immunohistochemistry (Paraffin).

Immunohistochemistry Analysis: A 1:1,000 dilution from a representative lot detected CBS in human liver and human pancreas tissue.

General description

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.

Immunogen

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

Other Notes

Concentration: Please refer to lot specific datasheet.

Physical form

Format: Purified

Quality

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.

Specificity

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

Target description

~61 kDa observed