General description
Cystathionine-β-synthase (CBS) also known as histone promoter control protein Hip4, is encoded by the gene mapped to human chromosome 21q22.3. CBS is a homotetramer containing 63kDa subunits. The encoded protein comprises C-terminal domain with a negative regulatory region, the middle domain with the catalytic core and heme-containing N-terminal domain.
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. (provided by RefSeq)
Immunogen
CBS (NP_000062, 1 a.a. ~ 100 a.a) partial recombinant protein with GST tag. MW of the GST tag alone is 26 KDa.
Sequence
MPSETPQAEVGPTGCPHRSGPHSAKGSLEKGSPEDKEAKEPLWIRPDAPSRCTWQLGRPASESPHHHTAPAKSPKILPDILKKIGDTPMVRINKIGKKFG
Biochem/physiol Actions
Cystathionine-β-synthase (CBS) catalyzes the first step in the transsulfuration pathway, where serine and homocysteine is converted to cystathionine and water. In addition, it also acts as a catalyst for various alternative reactions involved in the synthesis of hydrogen sulfide, a novel neuromodulator in the brain. Deficiency of the gene is associated with the development of an autosomal recessive disorder, homocystinuria.
Physical form
Solution in phosphate buffered saline, pH 7.4
Legal Information
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- UPC:
- 51182068
- Condition:
- New
- Availability:
- 3-5 Days
- Weight:
- 1.00 Ounces
- HazmatClass:
- No
- MPN:
- WH0000875M1-100UG
- Temperature Control Device:
- Yes
