Chemicals and Drugs

Uroporphyrinogen III Synthetase

An enzyme that catalyzes the cyclization of hydroxymethylbilane to yield UROPORPHYRINOGEN III and water. It is the fourth enzyme in the 8-enzyme biosynthetic pathway of HEME, and is encoded by UROS gene. Mutations of UROS gene result in CONGENITAL ERYTHROPOIETIC PORPHYRIA.

National Library of MedicineMedical Subject Headings2026

Structured Summary

Abstract

An enzyme that catalyzes the cyclization of hydroxymethylbilane to yield UROPORPHYRINOGEN III and water. It is the fourth enzyme in the 8-enzyme biosynthetic pathway of HEME, and is encoded by UROS gene. Mutations of UROS gene result in CONGENITAL ERYTHROPOIETIC PORPHYRIA.

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Synonyms

9 entry terms
  • Cosynthase
  • Uroporphyrinogen III Cosynthetase
  • Uroporphyrinogen Isomerase
  • Uroporphyrinogen-III Synthase
  • Cosynthetase, Uroporphyrinogen III
  • Isomerase, Uroporphyrinogen
  • Synthase, Uroporphyrinogen-III
  • Synthetase, Uroporphyrinogen III
  • Uroporphyrinogen III Synthase

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Aspects Covered

28 allowable subheadings

Indexed with the subheadings administration & dosage, adverse effects, analysis, antagonists & inhibitors, biosynthesis, blood, cerebrospinal fluid, chemical synthesis, chemistry, classification, 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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History Note

2005 (1975)

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Previous Indexing

  • Isomerases (1971-1974)
  • Porphobilinogen (1973-1974)

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AMA Style

References

  1. National Library of Medicine. Uroporphyrinogen III Synthetase. Medical Subject Headings (MeSH). 2026. Unique ID D014576. http://id.nlm.nih.gov/mesh/2026/D014576
  2. Uroporphyrinogen III Synthetase. In: Wikipedia. https://en.wikipedia.org/wiki/Uroporphyrinogen_III_synthase
  3. Uroporphyrinogen III Synthetase. In: Wikidata. https://www.wikidata.org/wiki/Q426504