The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
Protein concentration is above or equal to 0.05 mg/ml.
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Preparation and Storage
Stability and Storage
Shipped on dry ice. Upon delivery aliquot and store at -80ºC. Avoid freeze / thaw cycles.
pH: 8.00 Constituents: 0.31% Glutathione, 0.79% Tris HCl
pccA COMPLEMENTATION GROUP
PCCase subunit alpha
Propanoyl CoA:carbon dioxide ligase subunit alpha
Propanoyl-CoA:carbon dioxide ligase subunit alpha
Propionyl CoA carboxylase alpha chain
Propionyl CoA carboxylase alpha chain mitochondrial
Propionyl CoA carboxylase alpha polypeptide
Propionyl coenzyme A carboxylase alpha polypeptide
Propionyl-CoA carboxylase alpha chain
Metabolic intermediate metabolism; propanoyl-CoA degradation; succinyl-CoA from propanoyl-CoA: step 1/3.
Involvement in disease
Defects in PCCA are the cause of propionic 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, developmental retardation, and intolerance to protein.