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.

Year introduced: 2024

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Tree Number(s): D08.811.464.257.050.500

MeSH Unique ID: D000095562

Registry Number: EC 6.4.1.3

Entry Terms:

• Carboxylase, Propionyl-Coenzyme A
• Propionyl Coenzyme A Carboxylase
• Propionyl-CoA Carboxylase
• Propionyl CoA Carboxylase

Previous Indexing:

• Methylmalonyl-CoA Decarboxylase (1979-2023)

All MeSH Categories
Chemicals and Drugs Category
Enzymes and Coenzymes
Enzymes
Ligases
Carbon-Carbon Ligases
Acetyl-CoA Carboxylase
Propionyl-Coenzyme A Carboxylase