Anti-BCKDHA Rabbit Polyclonal Antibody for WB, IF/ICC, ELISA - P12694

Core Product Specifications and Parameters

Biological Background: BCKDHA Function and Localization

• BCKDHA encodes the alpha subunit of the mitochondrial branched-chain alpha-ketoacid dehydrogenase (BCKD) E1 component, also known as BCKDE1A or MSU1.
• Together with the beta subunit (BCKDHB), it forms the heterotetrameric E1 enzyme that catalyzes the rate-limiting decarboxylation of alpha-ketoacids derived from valine, leucine, and isoleucine.
• The BCKD complex is central to branched-chain amino acid catabolism, producing CO2 and acyl-CoA for energy production; defects in BCKDHA are associated with maple syrup urine disease (MSUD).
• The protein localizes to the mitochondrion matrix, where it functions as a thiamine pyrophosphate-dependent oxidoreductase.
• Post-translational modifications include acetylation and phosphorylation, and the protein binds potassium as a cofactor.
• Alternative splicing generates multiple transcript variants.

Experimental Guidance and Technical Tips

• The immunogen corresponds to amino acids 276-445 of human BCKDHA, a region that may confer specificity across species; cross-reactivity with mouse and rat is reported.
• For Western blot, a band near 50 kDa is expected; consider enriching mitochondrial fractions if signal is weak in whole-cell lysates.
• In immunocytochemistry/immunofluorescence, a mitochondrial staining pattern should be anticipated; co-staining with mitochondrial markers is recommended.
• ELISA applications may benefit from optimizing coating antigen and antibody pair orientation; validate in the relevant sample system.

CamelBio: Your One-Stop Sourcing Bridge

CamelBio supports diagnostic manufacturers and research laboratories with a comprehensive portfolio of IVD raw materials, including validated antibody pairs, high-quality polyclonal antibodies like this anti-BCKDHA reagent, and bulk ancillary reagents. For projects targeting inborn errors of metabolism such as maple syrup urine disease, CamelBio can also source rare antibodies, recombinant proteins, and provide technical consulting to accelerate assay development from concept to clinic.