| Field | Specification |
|---|---|
| Mfr No | |
| Clonality | |
| Host | |
| Immunogen | E.coli-derived human DBT recombinant protein (Position: K46-R462) was used as the immunogen for the BCKDH E2 antibody. |
| Isotype | |
| Product Type | |
| Purity | |
| Reactivity | |
| Storage | |
| Target | |
| UniProt # |
Overview
BCKDH 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.
Key elements and design rationale
- Target: E2
- Antibody details: Rabbit, Polyclonal (rabbit origin), isotype Rabbit IgG
- Format: Lyophilized
- Applications (as listed): WB, ICC/IF, IP, FACS, ELISA
Biological background
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
Research relevance and current trends
- Connecting protein-level changes to phenotype using orthogonal readouts (genetic perturbation, transcriptomics, imaging).
- Considering isoforms and post-translational regulation when interpreting protein-level changes.
- Comparing results across species and model systems with matched controls.
Common research applications
- Western blotting: compare relative abundance and activation-state changes across conditions.
- Immunofluorescence: visualize subcellular distribution and cell-to-cell heterogeneity.
- Flow cytometry: quantify target-positive populations and signal shifts at single-cell resolution.
- ELISA: support antibody-based quantification in assay formats where applicable.
Interpret changes in signal alongside appropriate controls and, when relevant, in parallel with total-protein or pathway readouts.
Notes for experimental interpretation
- Signal can reflect expression level, isoform composition, and post-translational state; interpret results in the context of your model system and stimuli.
- Species differences and sample matrices can influence epitope recognition; prioritize matched controls and orthogonal confirmation when feasible.
Antibody notes: Polyclonal antibodies recognize multiple epitopes, which can broaden the epitope footprint and may increase sensitivity in some contexts.
Customization & Add-ons: Can’t find the antibody you need—or require a custom format for your assay? We can help you source the best match or support custom antibody solutions for diverse research needs, including species and isotype selection, conjugations and labeling (e.g., HRP/AP, biotin, fluorophores), purification grade options (Protein A/G, affinity purified), formulation preferences (buffer selection, carrier-free, glycerol-free), custom concentrations and aliquoting, low-endotoxin options for cell-based work, and application-focused QC/validation support (project dependent). Click Talk to a Scientist to submit a request, email us at support@biohippo.com, or explore our Research Services for additional support—our team will follow up with feasibility details and next steps.
