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.,
Cytoplasm.
Highly expressed in liver (PubMed:10727430, PubMed:20305127). Also in muscle and lower expression in most tissues except heart, pituitary gland, spleen, thymus, and vascular tissue, where it is hardly detected (PubMed:20305127).
下载说明书
