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 associated 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 retardation and skeletal abnormalities.
应用类型
Western blot: ~1:1000, IHC: ~1:10–1:50, IF: ~1:10–1:50, FACS: ~1:10-1:50.
免疫原
This CBS antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide between 301-330 amino acids from the Central region of human CBS.