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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 fpropionic acidemia type I (PA-1) [MIM:606054]. 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.
For Research Use Only. Not for use in diagnostic procedures. Not for resale without express authorization.
Protein Aliases: 1700055P21Rik; coagulation factor V/VIII-homology domains protein 1; CUB, LCCL and coagulation factor V/VIII-homology domains protein 1; discoidin; Discoidin, CUB and LCCL domain-containing protein 2; Endothelial and smooth muscle cell-derived neuropilin-like protein
Gene Aliases: 1700055P21Rik; AW146002; CLCP1; DCBLD2; ESDN
UniProt ID: (Human) Q96PD2, (Mouse) Q91ZV3, (Rat) Q91ZV2
Entrez Gene ID: (Human) 131566, (Mouse) 73379, (Rat) 155696
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