Anti-BCKDHB Rabbit Polyclonal Antibody for WB, ELISA - P21953

Core Product Specifications and Parameters

Biological Background: BCKDHB Function and Localization

• BCKDHB encodes the beta subunit of the mitochondrial branched-chain alpha-ketoacid dehydrogenase (BCKD) complex E1 component, forming a heterotetramer with BCKDHA.
• The BCKD complex catalyzes the oxidative decarboxylation of alpha-ketoacids derived from valine, leucine, and isoleucine, producing CO2 and acyl-CoA for energy production.
• The E1 beta subunit participates in the first step of the reaction: decarboxylation of the alpha-ketoacid and formation of an enzyme-substrate intermediate, followed by reductive acylation mediated by the lipoylamide cofactor of the E2 subunit.
• The protein is localized to the mitochondrial matrix, consistent with its role in amino acid catabolism.
• Post-translational modification by acetylation has been identified, potentially regulating enzyme activity.
• Mutations in BCKDHB are associated with maple syrup urine disease (MSUD), a metabolic disorder characterized by accumulation of branched-chain amino acids.

Experimental Guidance and Technical Tips

• The immunogen corresponds to amino acids 183–392 of human BCKDHB, which may be used for epitope mapping and specificity analysis.
• Expected molecular weight in Western blot is approximately 43 kDa; validate in the relevant sample system.
• Cross-reactivity with mouse and rat is reported; consider for comparative studies across species.
• For ELISA applications, optimize coating and detection conditions using the recombinant immunogen as a positive control.

CamelBio: Your One-Stop Sourcing Bridge

This BCKDHB antibody supports research into mitochondrial metabolism, branched-chain amino acid catabolism, and maple syrup urine disease. CamelBio offers a streamlined sourcing experience for IVD raw materials—including validated antibody pairs, optimized monoclonal and polyclonal antibodies, bulk ancillary reagents, and rare-target procurement—backed by technical guidance from concept to clinic.