|Calculated MW||60587 Da|
|Positive Control||WB: Hela cell lysates, rh-CBS|
|Application & Usage||WB: 1-4 µg|
|Other Names||Beta-thionase, Serine sulfhydrase, Cystathionine beta-synthase|
|Formulation||In PBS pH 7.2, 0.01 % BSA, 0.03 % ProClin® and 50 % glycerol|
|Reconstitution & Storage||-20 °C|
|Precautions||Cystathionine β-Synthase Antibody is for research use only and not for use in diagnostic or therapeutic procedures.|
|Function||Hydro-lyase catalyzing the first step of the transsulfuration pathway, where the hydroxyl group of L-serine is displaced by L-homocysteine in a beta-replacement reaction to form L-cystathionine, the precursor of L-cysteine. This catabolic route allows the elimination of L-methionine and the toxic metabolite L- homocysteine (PubMed:23981774, PubMed:20506325, PubMed:23974653). Also involved in the production of hydrogen sulfide, a gasotransmitter with signaling and cytoprotective effects on neurons (By similarity).|
|Cellular Location||Cytoplasm. Nucleus|
|Tissue Location||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|
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Provided below are standard protocols that you may find useful for product applications.
Cystathionine β-synthase (CBS; E.C. 22.214.171.124) is a PLP-dependent enzyme which plays a central role in sulfur amino acid metabolism in eukaryotes. CBS catalyzes condensation between serine and homocysteine to generate cystathionine, which is then further processed by cystathionine γ-lyase to yield cysteine. The gene encoding CBS is essentially linked to the genetic disorders of homocystinuria and Down syndrome. Homocystinuria is an autosomal recessive disease, characterized by high plasma levels of homocysteine, with clinical manifestations including mental retardation, thromboembolism and connective tissue defects. In addition, CBS also mediates synthesis of hydrogen sulfide by catalyzing condensation between cysteine and homocysteine. CBS is highly expressed in the nervous system, liver and kidney and is responsible for up to 95% of the H2S production in the brain.
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