{"product_id":"bckdh-e2-antibody-dihydrolipoamide-branched-chain-transacylase-e2-dbt-bha17136283","title":"BCKDH E2 Antibody \/ Dihydrolipoamide branched chain transacylase E2 \/ DBT","description":"\u003ch2\u003eOverview\u003c\/h2\u003e\u003cp\u003eBCKDH E2 Antibody \/ Dihydrolipoamide branched chain transacylase E2 \/ DBT is a anti-E2 Rabbit antibody Polyclonal (rabbit origin) supplied in Lyophilized format. Recommended for workflows such as Western blot (WB), Immunocytochemistry (ICC), Immunofluorescence (IF), Immunoprecipitation (IP), Flow cytometry (FACS), ELISA with listed reactivity in Human, Mouse, Rat. Reported localization: Mitochondria.\u003c\/p\u003e\u003ch2\u003eKey elements and design rationale\u003c\/h2\u003e\u003cul\u003e\n\u003cli\u003e\n\u003cstrong\u003eTarget:\u003c\/strong\u003e E2\u003c\/li\u003e\n\u003cli\u003e\n\u003cstrong\u003eAntibody details:\u003c\/strong\u003e Rabbit, Polyclonal (rabbit origin), isotype Rabbit IgG\u003c\/li\u003e\n\u003cli\u003e\n\u003cstrong\u003eFormat:\u003c\/strong\u003e Lyophilized\u003c\/li\u003e\n\u003cli\u003e\n\u003cstrong\u003eApplications (as listed):\u003c\/strong\u003e WB, ICC\/IF, IP, FACS, ELISA\u003c\/li\u003e\n\u003c\/ul\u003e\u003ch2\u003eBiological background\u003c\/h2\u003e\u003cdiv\u003eBCKDH 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.\u003cbr\u003e\u003cbr\u003eBCKDH 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.\u003cbr\u003e\u003cbr\u003eFunctionally, 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.\u003cbr\u003e\u003cbr\u003eDefects 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.\u003cbr\u003e\u003cbr\u003eThe BCKDH E2 antibody from\u003c\/div\u003e\u003ch2\u003eResearch relevance and current trends\u003c\/h2\u003e\u003cul\u003e\n\u003cli\u003eConnecting protein-level changes to phenotype using orthogonal readouts (genetic perturbation, transcriptomics, imaging).\u003c\/li\u003e\n\u003cli\u003eConsidering isoforms and post-translational regulation when interpreting protein-level changes.\u003c\/li\u003e\n\u003cli\u003eComparing results across species and model systems with matched controls.\u003c\/li\u003e\n\u003c\/ul\u003e\u003ch2\u003eCommon research applications\u003c\/h2\u003e\u003cul\u003e\n\u003cli\u003e\n\u003cstrong\u003eWestern blotting:\u003c\/strong\u003e compare relative abundance and activation-state changes across conditions.\u003c\/li\u003e\n\u003cli\u003e\n\u003cstrong\u003eImmunofluorescence:\u003c\/strong\u003e visualize subcellular distribution and cell-to-cell heterogeneity.\u003c\/li\u003e\n\u003cli\u003e\n\u003cstrong\u003eFlow cytometry:\u003c\/strong\u003e quantify target-positive populations and signal shifts at single-cell resolution.\u003c\/li\u003e\n\u003cli\u003e\n\u003cstrong\u003eELISA:\u003c\/strong\u003e support antibody-based quantification in assay formats where applicable.\u003c\/li\u003e\n\u003c\/ul\u003e\u003cp\u003eInterpret changes in signal alongside appropriate controls and, when relevant, in parallel with total-protein or pathway readouts.\u003c\/p\u003e\u003ch2\u003eNotes for experimental interpretation\u003c\/h2\u003e\u003cul\u003e\n\u003cli\u003eSignal can reflect expression level, isoform composition, and post-translational state; interpret results in the context of your model system and stimuli.\u003c\/li\u003e\n\u003cli\u003eSpecies differences and sample matrices can influence epitope recognition; prioritize matched controls and orthogonal confirmation when feasible.\u003c\/li\u003e\n\u003c\/ul\u003e\u003cp\u003e\u003cstrong\u003eAntibody notes:\u003c\/strong\u003e Polyclonal antibodies recognize multiple epitopes, which can broaden the epitope footprint and may increase sensitivity in some contexts.\u003c\/p\u003e\u003c!-- Sources (internal): - UniProt search — UniProt — https:\/\/www.uniprot.org\/uniprotkb?query=E2 - NCBI Gene search — NCBI — https:\/\/www.ncbi.nlm.nih.gov\/gene\/?term=E2 - Ensembl search — Ensembl — https:\/\/www.ensembl.org\/Multi\/Search\/Results?q=E2 - Human Protein Atlas search — HPA — https:\/\/www.proteinatlas.org\/search\/E2 - PubMed (review) — NLM — https:\/\/pubmed.ncbi.nlm.nih.gov\/?term=E2+review --\u003e","brand":"NSJ Bioreagents","offers":[{"title":"Adding 0.2 ml of distilled water will yield a concentration of 500 ug\/ml \/ 100 ug","offer_id":53047324049773,"sku":"FY13381","price":449.0,"currency_code":"USD","in_stock":true}],"thumbnail_url":"\/\/cdn.shopify.com\/s\/files\/1\/0949\/7424\/7277\/files\/get_image_92fbfe98-2280-4454-82a3-4a54d4b2e57e.jpg?v=1782237115","url":"https:\/\/www.ebiohippo.com\/products\/bckdh-e2-antibody-dihydrolipoamide-branched-chain-transacylase-e2-dbt-bha17136283","provider":"BioHippo","version":"1.0","type":"link"}