The CBS Polyclonal Antibody (PACO39514) is a powerful tool for researchers studying CBS, a key enzyme involved in the important metabolic pathway of homocysteine metabolism. This antibody, generated in rabbits, exhibits high reactivity with human samples and has been validated for use in Western blot applications. By binding specifically to the CBS protein, this antibody allows for precise detection and analysis in a variety of cell types, making it a valuable asset for studies in biochemistry and metabolic research.
CBS, also known as cystathionine beta-synthase, plays a crucial role in the conversion of homocysteine to cystathionine, a process essential for maintaining proper levels of homocysteine in the body. Dysregulation of CBS activity has been linked to various metabolic disorders, making it a promising target for therapeutic interventions. By investigating the function of CBS, researchers can gain valuable insights into the mechanisms underlying these disorders and potentially develop new treatment strategies.
Antibody Name:
Cbs Antibody (PACO39514)
Antibody SKU:
PACO39514
Size:
50ug
Host Species:
Rabbit
Tested Applications:
ELISA, IF
Recommended Dilutions:
ELISA:1:2000-1:10000, IF:1:50-1:200
Species Reactivity:
Mouse
Immunogen:
Recombinant Mouse Cystathionine beta-synthase protein (2-561AA)
Immunofluorescent analysis of Hela cells using PACO39514 at dilution of 1:100 and Alexa Fluor 488-congugated AffiniPure Goat Anti-Rabbit IgG(H+L).
Background:
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. Also involved in the production of hydrogen sulfide, a gasotransmitter with signaling and cytoprotective effects on neurons.
Synonyms:
Cystathionine beta-synthase (EC 4.2.1.22) (Beta-thionase) (Serine sulfhydrase), Cbs
UniProt Protein Function:
CBS: Only known pyridoxal phosphate-dependent enzyme that contains heme. Important regulator of hydrogen sulfide, especially in the brain, utilizing cysteine instead of serine to catalyze the formation of hydrogen sulfide. Hydrogen sulfide is a gastratransmitter with signaling and cytoprotective effects such as acting as a neuromodulator in the brain to protect neurons against hypoxic injury. Defects in CBS are the cause of cystathionine beta- synthase deficiency (CBSD). CBSD is an enzymatic deficiency resulting in altered sulfur metabolism and homocystinuria. The clinical features of untreated homocystinuria due to CBS deficiency include myopia, ectopia lentis, mental retardation, skeletal anomalies resembling Marfan syndrome, and thromboembolic events. Light skin and hair can also be present. Biochemical features include increased urinary homocystine and methionine. Belongs to the cysteine synthase/cystathionine beta- synthase family. 2 isoforms of the human protein are produced by alternative splicing.Protein type: EC 4.2.1.22; Amino Acid Metabolism - glycine, serine and threonine; Other Amino Acids Metabolism - selenoamino acid; Amino Acid Metabolism - cysteine and methionine; LyaseCellular Component: cytoplasm; cytosol; nucleusMolecular Function: cystathionine beta-synthase activity; cysteine synthase activity; enzyme binding; heme binding; identical protein binding; lyase activity; metal ion binding; nitrite reductase (NO-forming) activity; oxygen binding; protein homodimerization activity; pyridoxal phosphate binding; ubiquitin protein ligase bindingBiological Process: amino acid biosynthetic process; blood vessel remodeling; cerebellum morphogenesis; cysteine biosynthetic process; cysteine biosynthetic process from serine; cysteine biosynthetic process via cystathionine; endochondral ossification; homocysteine metabolic process; maternal process involved in pregnancy; negative regulation of apoptosis; regulation of blood vessel size; regulation of cGMP metabolic process; regulation of JNK activity; response to folic acid; superoxide metabolic process; transsulfuration