Propionyl-Coenzyme A Carboxylase

Engelsk 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.

Svenska synonymer

Propionyl-CoA-karboxylas

Engelska synonymer

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