Beta-thionase; methylcysteine synthase; serine sulfhydrase

For Research Use Only. Not for use in diagnostic procedures.

E Coli

>=90% by SDS-PAGE

0.5 mg/ml in 50 mM Tris, 100 mM NaCl, pH 8.0 and 20% glycerol
Appearance: Liquid

At -20 degree C
Stable for at least 1 yr as supplied. Avoid repeated freeze and thaw cycles.

Small volumes of CBS recombinant protein 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.

Background: 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.

Proteins and Enzymes; Animal-Free Origin (AOF) Recombinant Proteins & EnzymesProteins and Enzymes; Proteins and Enzymes (A-Z)

SDS-PAGE

Recombinant human Cystathionine Beta Synthase can be used in inhibitor screening assays, activity studies, selectivity profiling, Western Blotting, ELISA, and numerouse similar applications.

61.9 kDa (1-551 aa, NT His Tag)

cystathionine-beta-synthase

cystathionine beta-synthase

Cystathionine beta-synthase

CBS_HUMAN

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. Multiple alternatively spliced transcript variants have been found for this gene. [provided by RefSeq, May 2010]

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; Other Amino Acids Metabolism - selenoamino acid; EC 4.2.1.22; Amino Acid Metabolism - glycine, serine and threonine; Lyase

Chromosomal Location of Human Ortholog: 21q22.3

Cellular Component: intracellular membrane-bound organelle; nucleolus; nucleus; cytosol

Molecular Function: identical protein binding; protein binding; protein homodimerization activity; enzyme binding; cystathionine beta-synthase activity; metal ion binding; ubiquitin protein ligase binding; heme binding; pyridoxal phosphate binding

Biological Process: cysteine biosynthetic process via cystathionine; homocysteine catabolic process; sulfur amino acid metabolic process; L-serine metabolic process; transsulfuration; cysteine biosynthetic process from serine; homocysteine metabolic process; L-serine catabolic process; L-cysteine catabolic process

Disease: Homocystinuria Due To Cystathionine Beta-synthase Deficiency

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