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propionyl-koenzým A karboxyláza
Subject h. propionyl-koenzým A karboxyláza Subject h. Propionyl-Coenzyme A Carboxylase Entry terms propionyl-KoA-karboxyláza
propionyl-CoA-karboxylázaEnglish X references Propionyl-CoA Carboxylase Scope note in English 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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