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.
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Număr Catalog 992-ET7109-21CategorieAfaceri și industrie > Știință și laboratorFurnizorHUABIOGentaurDimensiune100ulTipsingle
