Anti-DBT Rabbit Polyclonal Antibody for WB, IHC-P, IF/ICC, ELISA - P11182

Core Product Specifications and Parameters

Biological Background: DBT Function and Localization

• Lipoamide acyltransferase component of the branched-chain alpha-keto acid dehydrogenase complex (BCKDH), also known as DBT, BCKAD-E2, or BCOADC-E2.
• Core enzymatic subunit that accepts acyl groups from the E1 decarboxylase and transfers them to coenzyme A, driving the overall conversion of branched-chain α-keto acids to acyl-CoA and CO₂.
• Localized to the mitochondrial matrix, where it participates in the catabolism of the branched-chain amino acids valine, leucine, and isoleucine.
• Identified as a 52 kDa mitochondrial autoantigen in primary biliary cirrhosis (PBC).
• Post-translational modifications include lipoylation (essential for catalytic activity), acetylation, and phosphorylation.
• Mutations in the DBT gene cause maple syrup urine disease (MSUD), an autosomal recessive metabolic disorder characterized by accumulation of branched-chain amino acids.

Experimental Guidance and Technical Tips

• The immunogen covers amino acids 11–247 of human DBT (N-terminal region), enabling recognition of the full-length protein and potentially early processing intermediates.
• For Western blot, a specific band at ~53 kDa is expected; validate with appropriate positive and negative controls to confirm reactivity in your sample system.
• In IHC-P, antigen retrieval using heat-mediated citrate buffer (pH 6.0) may enhance detection; anticipate mitochondrial staining patterns.
• For IF/ICC, permeabilization is required to access the mitochondrial matrix target; consider co-staining with a mitochondrial marker.
• ELISA performance should be optimized with recombinant DBT or cell lysates; cross-reactivity with mouse and rat orthologs should be verified.

CamelBio: Your One-Stop Sourcing Bridge

CamelBio empowers diagnostic manufacturers and research teams with reliable access to validated antibodies, recombinant proteins, and ancillary reagents for metabolic and mitochondrial research. This anti-DBT polyclonal antibody supports studies in branched-chain amino acid catabolism and maple syrup urine disease, backed by our extensive supply network for rare targets and custom development solutions.