" /> Propionyl-Coenzyme A Carboxylase - CISMeF





Preferred Label : Propionyl-Coenzyme A Carboxylase;

MeSH 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.;

MeSH synonym : Carboxylase, Propionyl-Coenzyme A; Propionyl Coenzyme A Carboxylase; Propionyl-CoA Carboxylase; Propionyl CoA Carboxylase;

UNII : EC 6.4.1.3;

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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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05/05/2025


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