Anti-PCCA, ID (PCCA, Propionyl-CoA carboxylase alpha chain, mitochondrial, Propanoyl-CoA:carbon diox

The protein encoded by this gene is the alpha subunit of the heterodimeric mitochondrial enzyme Propionyl-CoA carboxylase. PCCA encodes the biotin-binding region of this enzyme. Mutations in either PCCA or PCCB (encoding the beta subunit) lead to an enzyme deficiency resulting in propionic acidemia. Multiple transcript variants encoding different isoforms have been found for this gene. Applications: Suitable for use in Western Blot, Immunohistochemistry, ELISA, Recommended Dilution: ELISA: 1:1,000, Western Blot: 1:100-500, Immunohistochemistry: 1:10-50, Storage and Stability: May be stored at 4°C for short-term only. Aliquot to avoid repeated freezing and thawing. Store at -20°C. Aliquots are stable for 12 months. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.