Detection Method:Sandwich
Test principle:This assay employs a two-site sandwich ELISA to quantitate CBS in samples. An antibody specific for CBS has been pre-coated onto a microplate. Standards and samples are pipetted into the wells and anyCBS present is bound by the immobilized antibody. After removing any unbound substances, a biotin-conjugated antibody specific for CBS is added to the wells. After washing, Streptavidin conjugated Horseradish Peroxidase (HRP) is added to the wells. Following a wash to remove any unbound avidin-enzyme reagent, a substrate solution is added to the wells and color develops in proportion to the amount of CBS bound in the initial step. The color development is stopped and the intensity of the color is measured.
Product Overview:Cystathionine Beta Synthase?acts as a homotetramer to catalyze the conversion of homocysteine to cystathionine, the first step in the transsulfuration pathway. The encoded protein is allosterically activated by adenosyl-methionine and uses pyridoxal phosphate as a cofactor. Defects in this gene can cause cystathionine beta-synthase deficiency (CBSD), which can lead to homocystinuria. Multiple alternatively spliced transcript variants have been found for this gene.The deduced 551-residue protein showed about 90% identity with the rat protein. Northern blot analysis identified a major 2.7-kb mRNA transcript.The gene is expressed as a 2.5-kb mRNA species mostly in liver and pancreas, with faint expression in brain, heart, kidney and lung. In addition, a 3.7-kb transcript was found in pancreas and liver.
