FIGURE: 1 / 6
For reconstitution, we recommend adding 100 µL distilled water to a final antibody concentration of about 1 mg/mL. To use this carrier-free antibody for conjugation experiments, we strongly recommend performing another round of desalting. (Zeba Spin Desalting Columns, 7KMWCO, 0.5 mL, Product # 89882)
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.
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Protein Aliases: 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 Coenzyme A carboxylase, alpha polypeptide; Propionyl-CoA carboxylase alpha chain, mitochondrial; RP11-151A6.1
Gene Aliases: PCCA
UniProt ID: (Human) P05165
Entrez Gene ID: (Human) 5095
Molecular Function: ligase