Anti-CBS Antibody, clone 9F3.2,100UG,MABS518,Sigma

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订货号 0BV7734
品牌型号 Sigma MABS518
货期 询货期
最小订货量 1件
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产品介绍 Product Description

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.
~61 kDa observed


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.


Biochem/physiol Actions

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


Physical form

Format: Purified


Analysis Note

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.


Other Notes

Concentration: Please refer to lot specific datasheet.


技术参数 Specifications
biological sourcemouse
Quality Segment100
conjugateunconjugated
antibody formpurified immunoglobulin
antibody product typeprimary antibodies
clone9F3.2, monoclonal
species reactivityhuman
technique(s)immunohistochemistry: suitable (paraffin),western blot: suitable
isotypeIgG2aκ
NCBI accession no.NP_001171479
UniProt accession no.P35520
shipped indry ice
target post-translational modificationunmodified
Gene Informationhuman ... CBS(875)
长度(mm)
宽度(mm)
高度(mm)
重量(kg)
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