Anti-PCCB Rabbit Polyclonal Antibody for WB and ELISA - P05166

Core Product Specifications and Parameters

Biological Background: PCCB Function and Localization

• PCCB encodes the beta subunit of propionyl-CoA carboxylase (PCC), a biotin-dependent mitochondrial enzyme essential for the catabolism of odd-chain fatty acids, cholesterol, and several amino acids (isoleucine, threonine, methionine, valine). Related references: PMID:15890657, PMID:6765947
• Within the PCC holoenzyme, the alpha subunit (PCCA) catalyzes the ATP-dependent carboxylation of the biotin cofactor, while the beta subunit (PCCB) transfers the activated carboxyl group from biotin to propionyl-CoA to generate (S)-methylmalonyl-CoA.
• PCCB also displays minor activity toward butyryl-CoA, converting it to ethylmalonyl-CoA, and can accept alternative substrates such as (2E)-butenoyl-CoA/crotonoyl-CoA. Related references: PMID:6765947
• The protein localizes to the mitochondrial matrix and contains a transit peptide for mitochondrial import.
• Post-translational modifications include acetylation and phosphorylation, which may regulate enzyme activity or stability.
• Mutations in PCCB are associated with propionic acidemia, a severe metabolic disorder characterized by the accumulation of toxic metabolites.
• Alternative splicing yields multiple transcript variants, potentially producing isoforms with distinct functional properties.

Experimental Guidance and Technical Tips

• Because the immunogen spans amino acids 260–539, the antibody likely recognizes a central to C-terminal epitope on PCCB; consider using mitochondrial-enriched fractions or whole-cell lysates from human or mouse tissues for Western blotting.
• For ELISA, test a dilution series of recombinant PCCB protein or tissue lysates to determine optimal antibody concentration.
• Validate band specificity by siRNA knockdown or using PCCB-deficient cell lines if available.
• The predicted molecular weight is 58 kDa, but post-translational modifications or precursor processing may shift the observed band.

CamelBio: Your One-Stop Sourcing Bridge

CamelBio stands as your comprehensive one-stop sourcing partner for IVD raw materials, offering validated antibody pairs, optimized polyclonal antibodies like this anti-PCCB antibody, bulk ancillary reagents, and rare target sourcing to accelerate your diagnostic and research workflows from concept to clinic. This antibody supports studies in mitochondrial metabolism, branched-chain amino acid catabolism, and inborn errors of metabolism such as propionic acidemia. For your complete raw-material needs, trust CamelBio's expert supply chain and technical support.