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.1) Ugarte, M., (er) Am. J. Hum. Genet. 81 (6) (2007) In press.
Immunogen
Antibody produced in rabbits immunized with a synthetic peptide corresponding a region of human PCCA.
Key Feature
Clonality
Polyclonal
Host Species
Rabbit
Tested Applications
ELISAWB
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 μg/mL, and HRP conjugated secondary antibody should be diluted 1:50,000 - 100,000.:
Antibody used in WB on Transfected 293T at 0.2-1 ug/ml.
Positive Control
1) Tranfected 293T Cell Lysate
Application Notes
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 μg/mL, and HRP conjugated secondary antibody should be diluted 1:50,000 - 100,000.:
Additional Information
Form
Liquid
Storage Instructions
For short periods of storage (days) store at 4˚C. For longer periods of storage, store PCCA antibody at -20˚C. As with any antibody avoid repeat freeze-thaw cycles.
Storage Buffer
Antibody is lyophilized in PBS buffer with 2% sucrose. Add 50 μL of distilled water. Final antibody concentration is 1 mg/mL.