|Application ||WB, IHC, IF, FC|
|Calculated MW||60587 Da|
|Positive Control||Western blot: Raji, Rat brain and cerebellum tissue lysate, IHC: human brain tissue, FACS: 293 cells, IF: 293 cells|
|Application & Usage||Western blot: ~1:1000, IHC: ~1:10–1:50, IF: ~1:10–1:50, FACS: ~1:10-1:50.|
|Other Names||CBS; Cystathionine beta-synthase; Beta-thionase; Serine sulfhydrase|
|Formulation||100 µl of antibody in PBS with 0.09% (W/V) sodium azide|
|Handling||The antibody solution should be gently mixed before use.|
|Reconstitution & Storage||-20 °C|
|Precautions||CBS Antibody (Center) 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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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.
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