PCCA is the alpha subunit of the heterodimeric mitochondrial enzyme Propionyl-CoA carboxylase. PCCA is 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.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. Two transcript variants encoding different isoforms have been found for this gene.
PCCA antibody can be used for detection of PCCA by ELISA at 1:312500. PCCA antibody can be used for detection of PCCA by western blot at 1 ?g/mL, and HRP conjugated secondary antibody should be diluted 1:50,000 - 100,000.
Antibody is purified by peptide affinity chromatography method.
Antibody is lyophilized in PBS buffer with 2% sucrose. Add 50 ?L of distilled water. Final antibody concentration is 1 mg/mL.
Aliquot and store at -20°C or below. Avoid multiple freeze-thaw cycles.
Type:
Antigen: PCCA
Clonality: Polyclonal
Clone:
Conjugation: Unconjugated
Epitope:
Host: Rabbit
Isotype:
Reactivity: Human
