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Substance Name: Cystathionine beta-Synthase
RN: 9023-99-8


  • 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
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Names and Synonyms

Name of Substance

  • Cystathionine beta-Synthase

MeSH Heading

  • Cystathionine beta-Synthase


  • beta Thionase
  • beta-Synthase, cystathionine
  • beta-Thionase
  • Cystathionine beta synthase
  • Cystathionine synthetase
  • Serine sulfhydrase
  • Sulfhydrase, serine
  • Synthetase, cystathionine

Systematic Name

  • L-Serine hydro-lyase (adding homocysteine)

Registry Numbers

CAS Registry Number

  • 9023-99-8

System Generated Number

  • 0009023998