Chemicals and Drugs

Homogentisate 1,2-Dioxygenase

A mononuclear Fe(II)-dependent oxygenase, this enzyme catalyzes the conversion of homogentisate to 4-maleylacetoacetate, the third step in the pathway for the catabolism of TYROSINE. Deficiency in the enzyme causes ALKAPTONURIA, an autosomal recessive disorder, characterized by homogentisic aciduria, OCHRONOSIS and ARTHRITIS. This enzyme was formerly characterized as EC 1.13.1.5 and EC 1.99.2.5.

National Library of MedicineMedical Subject Headings2026

Structured Summary

Abstract

A mononuclear Fe(II)-dependent oxygenase, this enzyme catalyzes the conversion of homogentisate to 4-maleylacetoacetate, the third step in the pathway for the catabolism of TYROSINE. Deficiency in the enzyme causes ALKAPTONURIA, an autosomal recessive disorder, characterized by homogentisic aciduria, OCHRONOSIS and ARTHRITIS. This enzyme was formerly characterized as EC 1.13.1.5 and EC 1.99.2.5.

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Synonyms

11 entry terms
  • Homogentisate Dioxygenase
  • Homogentisate Oxidase
  • Homogentisate Oxygenase
  • Homogentisic Acid Oxidase
  • 1,2-Dioxygenase, Homogentisate
  • Acid Oxidase, Homogentisic
  • Dioxygenase, Homogentisate
  • Homogentisate 1,2 Dioxygenase
  • Oxidase, Homogentisate
  • Oxidase, Homogentisic Acid
  • Oxygenase, Homogentisate

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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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History Note

2006(1969)

MeSH Record

Previous Indexing

  • Dioxygenases (1969-2005)

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References

  1. National Library of Medicine. Homogentisate 1,2-Dioxygenase. Medical Subject Headings (MeSH). 2026. Unique ID D050560. http://id.nlm.nih.gov/mesh/2026/D050560
  2. Homogentisate 1,2-Dioxygenase. In: Wikipedia. https://en.wikipedia.org/wiki/Homogentisate_1,2-dioxygenase
  3. Homogentisate 1,2-Dioxygenase. In: Wikidata. https://www.wikidata.org/wiki/Q24783466