Testing Data
Rabbit anti-Human Cystathionine beta-Synthase Polyclonal Antibody | anti-CBS antibody
Cystathionine beta-Synthase Antibody
In PBS pH 7.2; 0.01 % BSA; 0.03 % ProClin and 50 % glycerol
The antibody solution should be gently mixed before use.
Testing Data
Testing Data
Cystathionine beta-synthase (CBS; E.C. 4.2.1.22) is a PLP-dependent enzyme which plays a central role in sulfur amino acid metabolism in eukaryotes. CBS catalyzes condensation between serine and homocysteine to generate cystathionine; which is then further processed by cystathionine gamma-lyase to yield cysteine. The gene encoding CBS is essentially linked to the genetic disorders of homocystinuria and Down syndrome. Homocystinuria is an autosomal recessive disease; characterized by high plasma levels of homocysteine; with clinical manifestations including mental retardation; thromboembolism and connective tissue defects. In addition; CBS also mediates synthesis of hydrogen sulfide by catalyzing condensation between cysteine and homocysteine. CBS is highly expressed in the nervous system; liver and kidney and is responsible for up to 95% of the H2S production in the brain.
NCBI and Uniprot Product Information
NCBI Description
The protein encoded by this gene acts as a homotetramer to catalyze the conversion of homocysteine to cystathionine, the first step in the transsulfuration pathway. The encoded protein is allosterically activated by adenosyl-methionine and uses pyridoxal phosphate as a cofactor. Defects in this gene can cause cystathionine beta-synthase deficiency (CBSD), which can lead to homocystinuria. This gene is a major contributor to cellular hydrogen sulfide production. Multiple alternatively spliced transcript variants have been found for this gene. [provided by RefSeq, Feb 2016]
Uniprot Description
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: Amino Acid Metabolism - cysteine and methionine; Amino Acid Metabolism - glycine, serine and threonine; EC 4.2.1.22; Lyase; Other Amino Acids Metabolism - selenoamino acid
Chromosomal Location of Human Ortholog: 21q22.3
Cellular Component: cytoplasm; cytosol; nucleus
Molecular Function: cystathionine beta-synthase activity; enzyme binding; heme binding; identical protein binding; nitrite reductase (NO-forming) activity; oxygen binding; protein binding; protein homodimerization activity; pyridoxal phosphate binding; ubiquitin protein ligase binding
Biological Process: cysteine biosynthetic process; DNA protection; homocysteine catabolic process; homocysteine metabolic process; L-cysteine catabolic process; L-serine catabolic process; L-serine metabolic process; transsulfuration
Disease: Homocystinuria Due To Cystathionine Beta-synthase Deficiency
Research Articles on CBS
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Product Notes
The CBS cbs (Catalog #AAA845990) is an Antibody produced from Rabbit and is intended for research purposes only. The product is available for immediate purchase. The Cystathionine beta-Synthase Antibody reacts with Human and may cross-react with other species as described in the data sheet. AAA Biotech's Cystathionine beta-Synthase can be used in a range of immunoassay formats including, but not limited to, Western Blot (WB). WB: 1-4 ug. Researchers should empirically determine the suitability of the CBS cbs for an application not listed in the data sheet. Researchers commonly develop new applications and it is an integral, important part of the investigative research process. It is sometimes possible for the material contained within the vial of "Cystathionine beta-Synthase, Polyclonal Antibody" to become dispersed throughout the inside of the vial, particularly around the seal of said vial, during shipment and storage. We always suggest centrifuging these vials to consolidate all of the liquid away from the lid and to the bottom of the vial prior to opening. Please be advised that certain products may require dry ice for shipping and that, if this is the case, an additional dry ice fee may also be required.Precautions
All products in the AAA Biotech catalog are strictly for research-use only, and are absolutely not suitable for use in any sort of medical, therapeutic, prophylactic, in-vivo, or diagnostic capacity. By purchasing a product from AAA Biotech, you are explicitly certifying that said products will be properly tested and used in line with industry standard. AAA Biotech and its authorized distribution partners reserve the right to refuse to fulfill any order if we have any indication that a purchaser may be intending to use a product outside of our accepted criteria.Disclaimer
Though we do strive to guarantee the information represented in this datasheet, AAA Biotech cannot be held responsible for any oversights or imprecisions. AAA Biotech reserves the right to adjust any aspect of this datasheet at any time and without notice. It is the responsibility of the customer to inform AAA Biotech of any product performance issues observed or experienced within 30 days of receipt of said product. To see additional details on this or any of our other policies, please see our Terms & Conditions page.Item has been added to Shopping Cart
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