Anti-BCKDHB (E1B) Rabbit Monoclonal Antibody for WB, IHC-P, ELISA - P21953

Core Product Specifications and Parameters

Biological Background: BCKDHB Function and Localization

• BCKDHB encodes the beta subunit of the mitochondrial branched-chain alpha-keto acid dehydrogenase E1 component, which complexes with BCKDHA (alpha subunit) to form a heterotetrameric E1 enzyme.
• The BCKD complex catalyzes the oxidative decarboxylation of branched-chain alpha-keto acids derived from valine, leucine, and isoleucine, producing CO2 and acyl-CoA derivatives.
• This reaction is the first and rate-limiting step in the catabolism of branched-chain amino acids, connecting amino acid degradation to energy production and lipid synthesis.
• Alternative splicing generates multiple transcript variants (UniProt keyword), potentially regulating enzyme function in different tissues.
• The protein is localized to the mitochondrial matrix, consistent with its role in mitochondrial metabolism.
• Post-translational modifications include acetylation (keyword), which may modulate enzyme activity or stability.
• Mutations in BCKDHB are associated with Maple syrup urine disease (MSUD) type IB, a metabolic disorder characterized by accumulation of branched-chain amino acids and their keto acids.
• The protein is classified as an oxidoreductase (keyword), and its structure has been determined by 3D-structure analysis.

Experimental Guidance and Technical Tips

• The immunogen is a recombinant fusion protein spanning amino acids 51-392 of human BCKDHB. This region includes the mature protein sequence, and the antibody may recognize epitopes within this central domain.
• For Western blotting, the predicted molecular weight of BCKDHB is approximately 43 kDa (UniProt: 43122 Da). A band near this size may be observed; minor shifts due to post-translational modifications are possible.
• When performing immunohistochemistry (IHC-P), mitochondrial localization may result in granular cytoplasmic staining. Validate staining patterns with appropriate positive and negative control tissues.
• Cross-reactivity with mouse and rat orthologs is reported. Sequence alignment can be used to assess epitope conservation across species for quantitative applications.
• For ELISA applications, the antibody's binding site and affinity should be empirically determined using the recombinant immunogen or purified protein to establish assay conditions.
• Consider validating the antibody in relevant cell lines or tissue models for metabolic studies, such as liver or muscle tissues, where BCKDH activity is high.

CamelBio: Your One-Stop Sourcing Bridge

CamelBio helps diagnostic manufacturers and research labs accelerate metabolic disease assay development with a broad portfolio of IVD raw materials. Our anti-BCKDHB rabbit monoclonal antibody exemplifies our commitment to validated reagents for branched-chain amino acid metabolism and MSUD research. Complement your workflow with matched antibody pairs, bulk buffers, and custom rare-target sourcing services—all backed by CamelBio’s technical and regulatory guidance.