Chemicals and Drugs

Propionyl-Coenzyme A Carboxylase

Mitochondrial carboxylase that is involved in the catabolism of odd chain fatty acids and branched amino acids. Dysfunction of propionyl-CoA carboxylase leads to the inherited metabolic disorder PROPIONIC ACIDEMIA. In bacteria it is an ATP-dependent carboxylase in CO2 fixation reactions where propionyl-CoA is converted to (S)-methylmalonyl-CoA.

National Library of MedicineMedical Subject Headings2026

Structured Summary

Abstract

Mitochondrial carboxylase that is involved in the catabolism of odd chain fatty acids and branched amino acids. Dysfunction of propionyl-CoA carboxylase leads to the inherited metabolic disorder PROPIONIC ACIDEMIA. In bacteria it is an ATP-dependent carboxylase in CO2 fixation reactions where propionyl-CoA is converted to (S)-methylmalonyl-CoA.

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Synonyms

4 entry terms
  • Propionyl-CoA Carboxylase
  • Carboxylase, Propionyl-Coenzyme A
  • Propionyl CoA Carboxylase
  • Propionyl Coenzyme A Carboxylase

MeSH Record

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.

MeSH Record

History Note

2024; use Methylmalonyl-CoA Decarboxylase 2004-2023

MeSH Record

Previous Indexing

  • Methylmalonyl-CoA Decarboxylase (1979-2023)

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AMA Style

References

  1. National Library of Medicine. Propionyl-Coenzyme A Carboxylase. Medical Subject Headings (MeSH). 2026. Unique ID D000095562. http://id.nlm.nih.gov/mesh/2026/D000095562
  2. Propionyl-Coenzyme A Carboxylase. In: Wikipedia. https://en.wikipedia.org/wiki/Propionyl-CoA_carboxylase
  3. Propionyl-Coenzyme A Carboxylase. In: Wikidata. https://www.wikidata.org/wiki/Q415235