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.
Dilutions
WB 1:1000 - 1:3000
Format
Calculated MW
75kDa/77kDa/80kDa
Observed MW
80kDa
Immunogen
Recombinant fusion protein containing a sequence corresponding to amino acids 449-728 of human PCCA (NP_000273.2).
Modification
Unmodified
Storage
-20°C
Images
Western blot analysis of extracts of various cell lines, using PCCA antibody (144-09486) at 1:3000 dilution. Secondary antibody: HRP Goat Anti-Rabbit IgG (H+L) at 1:10000 dilution. Lysates/proteins: 25ug per lane. Blocking buffer: 3% nonfat dry milk in TBST. Detection: ECL Basic Kit. Exposure time: 1s.
Expiration:
12 months from the date of shipment when stored properly.
