Cystathionine beta-Synthase
MESHD003541
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.
Synonyms
- beta-Synthase, Cystathionine
- Cystathionine beta Synthase
- beta-Thionase
- beta Thionase
- Cystathionine Synthetase
- Synthetase, Cystathionine
- Serine Sulfhydrase
- Sulfhydrase, Serine
Source last updated 2026-05-24T15:27:57.172Z