Anti-BCKDHB Monoclonal Antibody for WB - P21953

Core Product Specifications and Parameters

Biological Background: BCKDHB Function and Localization

• BCKDHB (also known as E1B, BCKDE1B, BCKDH E1-beta) encodes the beta subunit of the mitochondrial branched-chain alpha-ketoacid dehydrogenase E1 component.
• Together with BCKDHA, it forms the heterotetrameric E1 subunit of the BCKD complex, which catalyzes the oxidative decarboxylation of alpha-ketoacids derived from valine, leucine, and isoleucine.
• The E1 subunit performs the first step: decarboxylation of the alpha-ketoacid to form an enzyme-product intermediate, followed by reductive acylation mediated by the E2 lipoylamide cofactor.
• BCKDHB localizes to the mitochondrial matrix, as indicated by its transit peptide and mitochondrial targeting.
• Post-translational modifications include acetylation, and alternative splicing produces multiple isoforms.
• Mutations in BCKDHB are associated with maple syrup urine disease (MSUD), a metabolic disorder affecting branched-chain amino acid catabolism.
• The protein has a molecular weight of approximately 43 kDa (mature form after transit peptide cleavage).

Experimental Guidance and Technical Tips

• The immunogen covers amino acids 51-392 of human BCKDHB, encompassing the mature protein after mitochondrial processing. For Western blot, a band around 43 kDa is expected; validate antibody specificity using positive controls (e.g., cells with high BCKDH activity) and consider using mitochondrial-enriched fractions.
• For ELISA, optimize coating and detection conditions with recombinant BCKDHB protein or cell lysates; the antibody may be used in direct or sandwich formats with careful titration.
• Cross-reactivity with mouse and rat has been reported, but performance should be confirmed in the relevant sample matrices.

CamelBio: Your One-Stop Sourcing Bridge

CamelBio provides diagnostic manufacturers, laboratories, and research institutes with one-stop access to IVD raw materials, including this anti-BCKDHB rabbit monoclonal antibody, which supports research into metabolic disorders like maple syrup urine disease. Our portfolio spans validated antibody pairs, optimized monoclonal/polyclonal antibodies, bulk ancillary reagents, and rare target sourcing, ensuring seamless integration into your assay development and manufacturing workflows.