Chemicals and Drugs

gamma-Butyrobetaine Dioxygenase

An enzyme that catalyzes the HYDROXYLATION of gamma-butyrobetaine to L-CARNITINE. It is the last enzyme in the biosynthetic pathway of L-CARNITINE and is dependent on alpha-ketoglutarate; IRON; ASCORBIC ACID; and OXYGEN.

National Library of MedicineMedical Subject Headings2026

Structured Summary

Abstract

An enzyme that catalyzes the HYDROXYLATION of gamma-butyrobetaine to L-CARNITINE. It is the last enzyme in the biosynthetic pathway of L-CARNITINE and is dependent on alpha-ketoglutarate; IRON; ASCORBIC ACID; and OXYGEN.

MeSH Record

Classification

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MeSH Record

Synonyms

17 entry terms
  • 4-Trimethylaminobutyrate, 2-Oxoglutarate Oxygen Oxidoreductase (3-Hydroxylating)
  • Butyrobetaine Hydroxylase
  • Trimethylaminobutyrate Hydroxylase
  • gamma-Butyrobetaine Hydroxylase
  • gamma-Butyrobetaine, 2-Oxoglutarate Dioxygenase
  • gamma-Butyrobetaine,2-Oxoglutarate Dioxygenase
  • 2-Oxoglutarate Dioxygenase gamma-Butyrobetaine
  • Dioxygenase gamma-Butyrobetaine, 2-Oxoglutarate
  • Dioxygenase, gamma-Butyrobetaine
  • Dioxygenase, gamma-Butyrobetaine,2-Oxoglutarate
  • Hydroxylase, Butyrobetaine
  • Hydroxylase, Trimethylaminobutyrate
  • Hydroxylase, gamma-Butyrobetaine
  • gamma Butyrobetaine Dioxygenase
  • gamma Butyrobetaine Hydroxylase
  • gamma Butyrobetaine, 2 Oxoglutarate Dioxygenase
  • gamma Butyrobetaine,2 Oxoglutarate Dioxygenase

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.

MeSH Record

History Note

2006(1980); use KETOGLUTARIC ACID 1973-1974, BETAINE 1972-1979, BUTYRATES 1974-1979, & AMINOBUTYRIC ACID 1970-1974

MeSH Hierarchy

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AMA Style

References

  1. National Library of Medicine. gamma-Butyrobetaine Dioxygenase. Medical Subject Headings (MeSH). 2026. Unique ID D050563. http://id.nlm.nih.gov/mesh/2026/D050563
  2. gamma-Butyrobetaine Dioxygenase. In: Wikidata. https://www.wikidata.org/wiki/Q3266583