蛋白名称
Cystathionine gamma-lyase (EC 4.4.1.1) (Cysteine-protein sulfhydrase) (Gamma-cystathionase)
免疫原
Synthesized peptide derived from part region of human protein
特异性
CGL Monoclonal Antibody detects endogenous levels of protein.
组成
Liquid in PBS containing 50% glycerol, and 0.02% sodium azide.
纯化工艺
The antibody was affinity-purified from mouse antiserum by affinity-chromatography using epitope-specific immunogen.
背景
This gene encodes a cytoplasmic enzyme in the trans-sulfuration pathway that converts cystathione derived from methionine into cysteine. Glutathione synthesis in the liver is dependent upon the availability of cysteine. Mutations in this gene cause cystathioninuria. Alternative splicing of this gene results in three transcript variants encoding different isoforms. [provided by RefSeq, Jun 2010],
功能
catalytic activity:L-cystathionine + H(2)O = L-cysteine + NH(3) + 2-oxobutanoate.,cofactor:Pyridoxal phosphate.,disease:Defects in CTH are the cause of cystathioninuria [MIM:219500]. CTH is an autosomal recessive phenotype characterized by abnormal accumulation of plasma cystathionine, leading to increased urinary excretion.,pathway:Amino-acid biosynthesis; L-cysteine biosynthesis; L-cysteine from L-homocysteine and L-serine: step 2/2.,PTM:Phosphorylated upon DNA damage, probably by ATM or ATR.,similarity:Belongs to the trans-sulfuration enzymes family.,subunit:Homotetramer.,
