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.
Specificity
Expected to react with both the Major (isoform 1) and the Minor (isoform 2) spliced variant.
Immunogen
Recombinant protein corresponding to the N-terminal of human CBS.
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.
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.
Target description
~61 kDa observed
Physical form
Format: Purified
Other Notes
Concentration: Please refer to lot specific datasheet.
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Storage Class
12 - Non Combustible Liquids
wgk_germany
WGK 1
flash_point_f
Not applicable
flash_point_c
Not applicable
Certificates of Analysis (COA)
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