Cystathionine beta-Synthase


Cystathionine Synthetase

Cystathionine beta Synthase

L-Serine hydro-lyase (adding homocysteine)

Serine Sulfhydrase

Sulfhydrase, Serine

Synthetase, Cystathionine

beta Thionase

beta-Synthase, Cystathionine


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