Lane 1: 293
Lane 2: 293
Lane 3: human liver tissue
Recombinant Rabbit monoclonal primary
CBS Recombinant Rabbit Monoclonal Antibody [JE40-99] (ET7109-21)
Recombinant protein within human cbs aa 400-550.
293, human liver tissue lysate, 293T.
Store at +4C after thawing. Aliquot store at -20C. Avoid repeated freeze / thaw cycles.
1*TBS (pH7.4), 0.05% BSA, 40% Glycerol. Preservative: 0.05% Sodium Azide.
Protein A purified.
AI047524 antibody; AI303044 antibody; Beta thionase antibody; Beta-thionase antibody; Cbs antibody; Cbs cystathionine beta-synthase antibody; CBS_HUMAN antibody; Cystathionine beta synthase antibody; Cystathionine beta-synthase antibody; EC 220.127.116.11 antibody; HIP 4 antibody; HIP4 antibody; Methylcysteine synthase antibody; MGC18856 antibody; MGC18895 antibody; MGC37300 antibody; OTTHUMP00000109416 antibody; OTTHUMP00000109418 antibody; Serine sulfhydrase antibody
Belongs to the cysteine synthase/cystathionine beta-synthase family.
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.
Strongly expressed in human liver and pancreas, with weaker expression in heart and brain, the cytoplasmic protein cystathionine b-synthase (CBS) operates in the first step of homocysteine transulfuration. CBS, which belongs to the cysteine synthase/cystathionine b-synthase family of proteins, catalyzes the formation of cystathionine from the thrombogenic amino acid homocysteine using pyridoxal phosphate cofactor. Allosteric activation by adenosyl-methionine regulates CBS activity. Deficiencies in CBS are assoc-iated with homocystinuria, a recessively inherited error in sulfur amino acid metabolism that affects many organs and tissues. Symptoms of homocytinuria include arteriosclerosis, thrombosis, dislocated optic lenses, mental retard-ation and skeletal abnormalities.