Anti-DBT Rabbit Monoclonal Antibody for WB, IHC-P, IP - P11182

Core Product Specifications and Parameters

Biological Background: DBT Function and Localization

• DBT encodes the E2 component (lipoamide acyltransferase) of the mitochondrial branched-chain alpha-keto acid dehydrogenase (BCKD) complex, also known as BCKAD-E2 or BCOADC-E2.
• The BCKD complex catalyzes the oxidative decarboxylation of branched-chain alpha-keto acids (derived from leucine, isoleucine, and valine) to produce acyl-CoA and CO₂. This E2 component transfers acyl groups from the E1 component to coenzyme A.
• DBT is localized to the mitochondrial matrix, where it participates in amino acid catabolism and energy production.
• DBT contains lipoyl-binding domains that are essential for its acyltransferase function and are subject to lipoylation.
• The protein undergoes post-translational modifications, including acetylation and phosphorylation, and is identified as a phosphoprotein.
• Mutations in DBT are associated with maple syrup urine disease (MSUD), an autosomal recessive metabolic disorder characterized by elevated branched-chain amino acids.
• DBT is also recognized as the 52 kDa mitochondrial autoantigen in primary biliary cirrhosis, highlighting its immunological relevance.
• The protein is highly conserved across mammalian species, with cross-reactivity demonstrated in human, mouse, and rat.

Experimental Guidance and Technical Tips

• The immunogen (amino acids 62–247) spans the lipoyl-binding domain and part of the catalytic core, which may contribute to epitope specificity.
• For western blot, expect a band at approximately 53 kDa; validate with appropriate controls in your sample system.
• For immunohistochemistry (paraffin-embedded), antigen retrieval may be required; optimize conditions for your tissue type.
• Immunoprecipitation and ELISA applications have been reported; consider titration for optimal signal-to-noise ratio.
• Due to high sequence homology across human, mouse, and rat, cross-reactivity is expected, but verify in your experimental context.

CamelBio: Your One-Stop Sourcing Bridge

CamelBio provides diagnostic manufacturers and research labs with direct access to this high-quality anti-DBT antibody, along with a comprehensive portfolio of IVD raw materials. As a dedicated sourcing partner for mitochondrial metabolism and metabolic disease research, CamelBio offers validated antibody pairs, recombinant proteins, and custom reagent solutions to streamline assay development from concept to clinic.