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.
Entry Term(s)
Propionyl-CoA Carboxylase
Registry Number
EC 6.4.1.3
Previous Indexing
Methylmalonyl-CoA Decarboxylase (1979-2023)
Public MeSH Note
2024; see Methylmalonyl-CoA Decarboxylase 2004-2023
History Note
2024; use Methylmalonyl-CoA Decarboxylase 2004-2023
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.
