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This protein is a key enzyme in the catabolic pathway of odd-chain fatty acids, isoleucine, threonine, methionine, and valine. It is probably a dodecamer composed of six biotin-containing alpha subunits and six beta subunits. The protein is located in the mitochondrial matrix. Defects in PCCA are the cause o propionic acidemia type I (PA-1). PA-1 is a life-threatening disease characterized by episodic vomiting, lethargy and ketosis, neutropenia, periodic thrombocytopenia, hypogammaglobulinemia, develop-mental retardation, and intolerance to protein.
C79630; Pcca; pccA complementation group; PCCase alpha subunit; PCCase subunit alpha; propanoyl-CoA:carbon dioxide ligase alpha subunit; propanoyl-CoA:carbon dioxide ligase subunit alpha; propionyl CoA carboxylase, alpha polypeptide; propionyl CoA-carboxylase alpha; Propionyl Coenzyme A carboxylase alpha polypeptide; propionyl Coenzyme A carboxylase, alpha polypeptide; propionyl-CoA carboxylase alpha chain, mitochondrial; propionyl-CoA carboxylase alpha subunit; propionyl-Coenzyme A carboxylase, alpha polypeptide; RP11-151A6.1
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