Chemicals and Drugs

Cystathionine beta-Synthase

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.

National Library of MedicineMedical Subject Headings2026

Structured Summary

Abstract

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.

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Classification

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See Also

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Synonyms

8 entry terms
  • Cystathionine Synthetase
  • Serine Sulfhydrase
  • beta-Thionase
  • Cystathionine beta Synthase
  • Sulfhydrase, Serine
  • Synthetase, Cystathionine
  • beta Thionase
  • beta-Synthase, Cystathionine

MeSH Record

Aspects Covered

29 allowable subheadings

Indexed with the subheadings administration & dosage, adverse effects, analysis, antagonists & inhibitors, biosynthesis, blood, cerebrospinal fluid, chemical synthesis, chemistry, classification, deficiency, drug effects, economics, genetics, history, immunology, isolation & purification, metabolism, pharmacokinetics, pharmacology, physiology, poisoning, radiation effects, standards, supply & distribution, therapeutic use, toxicity, ultrastructure, urine.

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Indexing Annotation

/defic: consider also HOMOCYSTINURIA or HYPERHOMOCYSTEINEMIA

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History Note

1991(1975)

MeSH Record

Previous Indexing

  • Homocysteine (1973-1974)
  • Hydro-Lyases (1973-1974)
  • Serine (1973-1974)

MeSH Hierarchy

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AMA Style

References

  1. National Library of Medicine. Cystathionine beta-Synthase. Medical Subject Headings (MeSH). 2026. Unique ID D003541. http://id.nlm.nih.gov/mesh/2026/D003541
  2. Cystathionine beta-Synthase. In: Wikidata. https://www.wikidata.org/wiki/Q24767109