Propionyl-Coenzyme A Carboxylase

**Definition:** 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.

**Tree numbers:** - D08.811.464.257.050.500

**Synonyms:** - Propionyl-CoA Carboxylase