
The protein encoded by this gene is the alpha subunit ofthe heterodimeric mitochondrial enzyme Propionyl-CoA carboxylase.PCCA encodes the biotin-binding region of this enzyme. Mutations ineither PCCA or PCCB (encoding the beta subunit) lead to an enzymedeficiency resulting in propionic acidemia. Multiple transcriptvariants encoding different isoforms have been found for this gene.
The stated application concentrations are suggested starting points. Titration of the PCCA antibody may be required due to differences in protocols and secondary/substrate sensitivity.
A portion of amino acids 362-390 from the human protein were used as the immunogen for the PCCA antibody.
Aliquot the PCCA antibody and store frozen at -20oC or colder. Avoid repeated freeze-thaw cycles.