Anti-PCCase beta Antibody

This rabbit polyclonal antibody recognizes the beta subunit of human propionyl-CoA carboxylase (PCCase beta), a biotin-dependent mitochondrial enzyme that catalyzes the ATP-dependent carboxylation of propionyl-CoA to D-methylmalonyl-CoA during the catabolism of odd-chain fatty acids and the breakdown of branched-chain amino acids including valine, isoleucine, methionine, and threonine. The beta chain carries the biotin carboxylase activity of the heterododecameric holoenzyme, while the alpha subunit provides the carboxyltransferase function. Mutations in the PCCB gene cause propionic acidemia, an autosomal recessive organic acidemia characterized by accumulation of propionyl-CoA and related metabolites, which makes detection of the beta chain relevant for metabolic disorder research and studies of mitochondrial enzyme biogenesis.

Supplied as an unconjugated antiserum at 100 µL, the reagent is validated for Western blot, immunohistochemistry, and immunofluorescence workflows on human cells and tissues. The polyclonal format offers multi-epitope recognition, useful in denaturing gel separations where conformational epitopes are lost and in aldehyde-fixed specimens where accessibility of the mitochondrial target depends on detergent-mediated permeabilization. Researchers commonly use this reagent alongside alpha-subunit antibodies to assess holoenzyme assembly, subcellular localization, and steady-state levels in patient-derived fibroblasts, lymphoblastoid cell lines, and induced pluripotent stem cell-derived hepatocyte or neuronal models relevant to organic acidemia.

The product is offered through Antibodies.com and ships in a vial format suitable for aliquoting upon arrival to minimize repeated freeze-thaw cycles. Store stock material at -20 °C; working dilutions can be refrigerated for short-term use. For immunofluorescence applications, fixation in 4% paraformaldehyde followed by Triton X-100 permeabilization provides a common starting point for visualizing the mitochondrial distribution of the enzyme. Users should optimize blocking conditions and antibody dilution empirically based on their sample type and detection system.