Full Product Name
CBS Antibody
Product Gene Name
anti-CBS antibody
[Similar Products]
Research Use Only
For Research Use Only. Not for use in diagnostic procedures.
3D Structure
ModBase 3D Structure for P35520
Species Reactivity
Human, Mouse, Rat
Concentration
1.0mg/ml (lot specific)
Other Notes
Small volumes of anti-CBS antibody vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.
Applications Tested/Suitable for anti-CBS antibody
Western Blot (WB), Immunohistochemistry (IHC)
NCBI/Uniprot data below describe general gene information for CBS. It may not necessarily be applicable to this product.
NCBI Accession #
NP_000062.1
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NCBI GenBank Nucleotide #
NM_000071.2
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UniProt Primary Accession #
P35520
[Other Products]
UniProt Secondary Accession #
Q99425; Q9BWC5; B2R993; D3DSK4[Other Products]
UniProt Related Accession #
P35520[Other Products]
Molecular Weight
61,863 Da
NCBI Official Full Name
cystathionine beta-synthase isoform 1
NCBI Official Synonym Full Names
cystathionine-beta-synthase
NCBI Official Symbol
CBS [Similar Products]
NCBI Official Synonym Symbols
HIP4
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NCBI Protein Information
cystathionine beta-synthase
UniProt Protein Name
Cystathionine beta-synthase
UniProt Synonym Protein Names
Beta-thionase; Serine sulfhydrase
Protein Family
Cystathionine beta-synthase
UniProt Gene Name
CBS [Similar Products]
NCBI Summary for CBS
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 Comments for CBS
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; cysteine 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; cysteine biosynthetic process from serine; 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
1. results indicate that binding of SAM to regulatory and stabilizing sites in CBS may have evolved to display an exquisite thermodynamic and structural specificity towards SAM as well as the ability to transduce the allosteric signal responsible for CBS activation.
Precautions
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