Anti-Cystathionine beta-synthase CBS Antibody Picoband® Fluoro647 Conjugated

Cystathionine-β-synthase, also known as CBS, is an enzyme that in humans is encoded by the CBS gene. It is mapped to 21q22.3 and contains 23 exons, ranging in size from 42 to 299 bp. CBS catalyzes the first step of the transsulfuration pathway, from homocysteine to cystathionine. It uses the cofactor pyridoxal-phosphate (PLP) and can be allosterically regulated by effectors such as the ubiquitous cofactor S-adenosyl-L-methionine (adoMet) . This enzyme belongs to the family of lyases, to be specific, the hydro-lyases, which cleave carbon-oxygen bonds. CBS is a multidomain enzyme composed of an N-terminal enzymatic domain and two CBS domains. The CBS gene is the most common locus for mutations associated with homocystinuria.

E.coli-derived human CBS recombinant protein (Position: A331-K551) . Human CBS shares 83% amino acid (aa) sequence identity with both mouse and rat CBS.

In the adult strongly expressed in liver and pancreas, some expression in heart and brain, weak expression in lung and kidney. In the fetus, expressed in brain, liver and kidney.

Only known pyridoxal phosphate-dependent enzyme that contains heme. Important regulator of hydrogen sulfide, especially in the brain, utilizing cysteine instead of serine to catalyze the formation of hydrogen sulfide. Hydrogen sulfide is a gastratransmitter with signaling and cytoprotective effects such as acting as a neuromodulator in the brain to protect neurons against hypoxic injury (By similarity) . .

1. Munke, M., Kraus, J., Watkins, P., Tanzi, R., Gusella, J., Millington Ward, A., Watson, M., Francke, U.Homocys 2. tinuria gene on human chromosome 21 mapped with cloned cystathionine beta-synthase probe and in situ hybridization of other chromosome 21 probes. (Abstract) Cytogenet. Cell Genet. 40: 706-707, 1985. 3. Kraus, J. P., Oliveriusova, J., Sokolova, J., Kraus, E., Vlcek, C., de Franchis, R., Maclean, K. N., Bao, L., Bukovska, G., Patterson, D., Paces, V., Ansorge, W., Kozich, V.The human cystathionine beta-synthase (CBS) gene: complete sequence, alternative splicing, and polymorphisms.Genomics 52: 312-324, 1998. 4. Kruger, W. D., Cox, D. R.A yeast system for expression of human cystathionine beta-synthase: structural and functional conservation of the human and yeast genes.Proc. Nat. Acad. Sci. 91: 6614-6618, 1994.