产品说明
一般描述
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)
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.
免疫原
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
生化/生理作用
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.
外形
Solution in phosphate buffered saline, pH 7.4
法律信息
GenBank is a registered trademark of United States Department of Health and Human Services
免责声明
Unless otherwise stated in our catalog or other company documentation accompanying the product(s), our products are intended for research use only and are not to be used for any other purpose, which includes but is not limited to, unauthorized commercial uses, in vitro diagnostic uses, ex vivo or in vivo therapeutic uses or any type of consumption or application to humans or animals.
基本信息
NACRES | NA.41 |
产品性质
质量水平 | 100 |
生物来源 | mouse |
偶联物 | unconjugated |
抗体形式 | purified immunoglobulin |
antibody product type | primary antibodies |
克隆 | 3E1, monoclonal |
形式 | buffered aqueous solution |
species reactivity | human |
technique(s) | immunohistochemistry (formalin-fixed, paraffin-embedded sections): suitable immunoprecipitation (IP): suitable indirect ELISA: suitable western blot: 1-5 μg/mL |
同位素/亚型 | IgG2aκ |
GenBank® accession no. | NM_000071 |
UniProt登记号 | P35520 |
运输 | dry ice |
储存温度 | −20℃ |
Gene Information | human ... CBS(875) |
安全信息
储存分类代码 | 12 - Non Combustible Liquids |
闪点(F) | Not applicable |
闪点(C) | Not applicable |
个人防护装备 | Eyeshields, Gloves, multi-purpose combination respirator cartridge (US) |
Sigma-Aldrich