蛋白名称
Cystathionine gamma-lyase (Cysteine-protein sulfhydrase) (Gamma-cystathionase)
组成
PBS, 50% glycerol, 0.05% Proclin 300, 0.05%BSA
来源
Monoclonal, Rabbit,IgG
稀释比例
IHC 1:200-1:1000; WB 1:2000-1:10000; IF 1:200-1:1000; ELISA 1:5000-1:20000; IP 1:50-1:200; Note: For IHC, we suggest antigen retrieval with TE buffer pH 9.0
储存
-15°C to -25°C/1 year(Do not lower than -25°C)
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.,
