- Tel: 858.663.9055
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Email: info@nsjbio.com
- Tel: 858.663.9055
- Email: info@nsjbio.com
BCKDH E2 Antibody / DBT Antibody recognizes dihydrolipoamide branched-chain transacylase E2, a mitochondrial enzyme encoded by the DBT gene. Also known as BCKAD-E2, BCKADE2 and E2B, DBT is the E2 component of the branched-chain alpha-keto acid dehydrogenase complex. This multienzyme complex catalyzes an irreversible step in the degradation of the branched-chain amino acids leucine, isoleucine and valine. DBT is synthesized as a nuclear-encoded precursor and transported into mitochondria, where it participates in the oxidative decarboxylation of branched-chain alpha-keto acids.
The BCKDH complex contains multiple copies of three catalytic components designated E1, E2 and E3. DBT forms the E2 core and functions as a dihydrolipoamide acyltransferase, accepting acyl groups generated by the E1 component and transferring them to coenzyme A. Lipoyl domains within DBT provide flexible reaction centers that facilitate movement of metabolic intermediates among the catalytic components of the complex. A BCKDH E2 Antibody can therefore support research examining the organization and function of this mitochondrial enzyme complex and the regulation of branched-chain amino acid catabolism.
BCKDH activity is tightly regulated to coordinate branched-chain amino acid utilization with cellular metabolic requirements. Impaired DBT function disrupts oxidative metabolism of branched-chain alpha-keto acids and can result in accumulation of branched-chain amino acids and their corresponding keto acids. Pathogenic variants in DBT are associated with maple syrup urine disease type II, reflecting the essential contribution of the E2 component to BCKDH complex activity. A DBT Antibody is therefore relevant to research investigating mitochondrial metabolism, branched-chain amino acid homeostasis and molecular mechanisms underlying defects in BCKDH function.
DBT is also recognized as a mitochondrial autoantigen in primary biliary cholangitis, where immune responses can target lipoylated components of mitochondrial dehydrogenase complexes. This provides an additional connection between BCKDH E2 biology, mitochondrial proteins and autoimmune research. More broadly, regulation of branched-chain amino acid catabolism is relevant to metabolic adaptation and cellular energy homeostasis. NSJ Bioreagents provides this polyclonal reagent for researchers investigating DBT and mitochondrial metabolism. A BCKDH E2 Antibody provides a useful tool for studying DBT, BCKDH complex biology and branched-chain amino acid metabolism.
This BCKDH E2 Antibody is part of our Metabolism Antibodies collection.
Optimal dilution of the BCKDH E2 Antibody / DBT Antibody should be determined by the researcher.
E.coli-derived human DBT recombinant protein (Position: K46-R462) was used as the immunogen for the BCKDH E2 antibody.
After reconstitution, the BCKDH E2 antibody can be stored for up to one month at 4oC. For long-term, aliquot and store at -20oC. Avoid repeated freezing and thawing.
DBT antibody, BCKAD-E2 antibody, BCKADE2 antibody, BCATE2 antibody, BCOADC-E2 antibody, E2B antibody, Dihydrolipoamide branched chain transacylase E2 antibody
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