Cystathionine beta-Synthase
Synonyms
Cystathionine Synthetase
Cystathionine beta Synthase
L-Serine hydro-lyase (adding homocysteine)
Serine Sulfhydrase
Sulfhydrase, Serine
Synthetase, Cystathionine
beta Thionase
beta-Synthase, Cystathionine
beta-Thionase
A multifunctional pyridoxal phosphate enzyme. In the second stage of cysteine biosynthesis it catalyzes the reaction of homocysteine with serine to form cystathionine with the elimination of water. Deficiency of this enzyme leads to HYPERHOMOCYSTEINEMIA and HOMOCYSTINURIA. EC 4.2.1.22.
See Also