Anti-PCCB Rabbit Monoclonal Antibody [KO Validated] for WB, IHC-P, IF/ICC, ELISA - P05166

Core Product Specifications and Parameters

Biological Background: PCCB (Propionyl-CoA Carboxylase Beta Chain) Function and Localization

• The PCCB gene encodes the beta subunit of propionyl-CoA carboxylase (PCC), a mitochondrial biotin-dependent enzyme; alternative splicing yields multiple isoforms.
• PCC catalyzes the carboxylation of propionyl-CoA to D-methylmalonyl-CoA, a key step in the catabolism of odd-chain fatty acids and branched-chain amino acids (isoleucine, threonine, methionine, valine). Related references: PMID:15890657 PMID:6765947
• Within the holoenzyme, the alpha subunit mediates ATP-dependent biotin carboxylation, while the beta subunit transfers the carboxyl group from biotin to propionyl-CoA (By similarity).
• The enzyme also exhibits activity toward butyryl-CoA, generating ethylmalonyl-CoA, and may act on crotonoyl-CoA as an alternative substrate (By similarity). Related references: PMID:6765947
• PCCB localizes exclusively to the mitochondrial matrix.
• The protein undergoes acetylation and phosphorylation and contains a mitochondrial transit peptide; mutations in PCCB are linked to propionic acidemia, an autosomal recessive metabolic disorder.

Experimental Guidance and Technical Tips

• This antibody has been validated using knockout (KO) models; include appropriate positive and negative controls in your experiments to confirm specificity.
• For Western blotting, the target protein runs at ~58 kDa; use a 10% or 12% SDS-PAGE gel and optimize transfer conditions for medium-sized proteins.
• In immunohistochemistry (IHC-P) and immunofluorescence (IF/ICC), verify staining in mitochondria-rich tissues or cell lines with known PCCB expression, using mitochondrial markers as colocalization references.
• The immunogen spans amino acids 260–539 of human PCCB, corresponding to a region likely involved in subunit interactions; this may help detect both native and denatured forms across applications.
• When setting up ELISA assays, consider testing a broad dilution range and using recombinant PCCB protein as a standard to determine optimal working concentrations.

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CamelBio supports diagnostic manufacturers and research laboratories with a full range of IVD raw materials, from validated monoclonal antibodies—such as this anti-PCCB product—to bulk ancillary reagents, helping you streamline assay development for metabolic and mitochondrial disease research. Our one-stop platform facilitates procurement of optimized antibody pairs, rare-target immunoreagents, and technical backup, accelerating your path from concept to clinical application. Whether you need a single specialized antibody or a complete raw-material panel, CamelBio acts as your reliable sourcing bridge.