深圳欣博盛生物科技有限公司

BCKDH E2 Antibody / Dihydrolipoamide branched chain transacylase E2 / DBT

BCKDH E2 antibody detects Dihydrolipoamide branched chain transacylase E2, the core component of the branched-chain alpha-keto acid dehydrogenase (BCKDH) complex encoded by the DBT gene on chromosome 1p31.3. This mitochondrial enzyme catalyzes a critical step in the oxidative decarboxylation of branched-chain amino acids (BCAAs)-leucine, isoleucine, and valine-linking amino acid catabolism to energy metabolism. The BCKDH E2 subunit serves as the central scaffold of the multienzyme complex, coordinating interactions with E1 (branched-chain alpha-keto acid decarboxylase) and E3 (dihydrolipoamide dehydrogenase) components to facilitate efficient substrate channeling within mitochondria.

BCKDH E2 belongs to the 2-oxo acid dehydrogenase family and forms a 24-mer cubic core structure that anchors multiple copies of the peripheral E1 and E3 enzymes. Each E2 subunit contains lipoyl domains that shuttle reaction intermediates between catalytic sites, an inner-core acyltransferase domain, and flexible linker regions that enable conformational movement during catalysis. The enzyme's lipoyl-lysine cofactors play a pivotal role in transferring acyl groups to CoA, producing acyl-CoA derivatives for entry into the tricarboxylic acid (TCA) cycle. Co-localization studies confirm mitochondrial matrix localization, consistent with its role in oxidative metabolism.

Functionally, BCKDH E2 catalyzes the acyl transfer step of BCAA degradation, converting alpha-ketoacid intermediates into corresponding acyl-CoA products and releasing carbon dioxide. This process provides both energy and metabolic intermediates for biosynthetic pathways. BCKDH E2 activity is tightly regulated by phosphorylation through BCKDH kinase (BCKDK) and dephosphorylation by PPM1K, ensuring proper response to nutrient availability and metabolic stress. In muscle and liver, BCKDH E2 contributes to nitrogen balance, mitochondrial energy generation, and regulation of plasma amino acid levels.

Defects in the DBT gene encoding BCKDH E2 cause Maple Syrup Urine Disease (MSUD) type II, characterized by accumulation of branched-chain amino acids and their ketoacid derivatives, leading to neurological dysfunction and metabolic crisis. Reduced BCKDH E2 function disrupts mitochondrial oxidative decarboxylation, resulting in toxic metabolite buildup. Conversely, increased BCKDH activity enhances amino acid catabolism under fasting or exercise conditions. Pathway associations include branched-chain amino acid degradation, acetyl-CoA biosynthesis, and mitochondrial energy metabolism. BCKDH E2 is highly expressed in liver, skeletal muscle, heart, and brain, reflecting its metabolic importance in energy-demanding tissues.

The BCKDH E2 antibody from NSJ Bioreagents is an ideal reagent for research into amino acid metabolism, mitochondrial enzymology, and inherited metabolic disorders.

Human, Mouse, Rat

Rabbit

WB, ICC/IF, IP, FACS, ELISA

Rabbit IgG

E.coli-derived human DBT recombinant protein (Position: K46-R462) was used as the immunogen for the BCKDH E2 antibody.

Polyclonal (rabbit origin)

Immunogen affinity purified

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.

Lyophilized

Adding 0.2 ml of distilled water will yield a concentration of 500 ug/ml

美国