| Host: | Rabbit |
| Applications: | WB/IF/ICC/ELISA |
| Reactivity: | Human/Mouse/Rat |
| Note: | STRICTLY FOR FURTHER SCIENTIFIC RESEARCH USE ONLY (RUO). MUST NOT TO BE USED IN DIAGNOSTIC OR THERAPEUTIC APPLICATIONS. |
| Clonality : | Polyclonal |
| Conjugation: | Unconjugated |
| Isotype: | IgG |
| Formulation: | PBS with 0.02% Sodium Azide, 50% Glycerol, pH 7.3. |
| Purification: | Affinity purification |
| Concentration: | Lot specific |
| Dilution Range: | WB:1:500-1:1000 IF/ICC:1:50-1:200 ELISA:Recommended starting concentration is 1 Mu g/mL. Please optimize the concentration based on your specific assay requirements. |
| Storage Instruction: | Store at-20°C for up to 1 year from the date of receipt, and avoid repeat freeze-thaw cycles. |
| Gene Symbol: | BCKDHA |
| Gene ID: | 593 |
| Uniprot ID: | ODBA_HUMAN |
| Immunogen Region: | 276-445 |
| Specificity: | Recombinant fusion protein containing a sequence corresponding to amino acids 276-445 of human BCKDHA (NP_000700.1). |
| Immunogen Sequence: | SEQYRGDGIAARGPGYGIMS IRVDGNDVFAVYNATKEARR RAVAENQPFLIEAMTYRIGH HSTSDDSSAYRSVDEVNYWD KQDHPISRLRHYLLSQGWWD EEQEKAWRKQSRRKVMEAFE QAERKPKPNPNLLFSDVYQE MPAQLRKQQESLARHLQTYG EHYPLDHFDK |
| Post Translational Modifications | Phosphorylated at Ser-337 by BCKDK and dephosphorylated by protein phosphatase PPM1K. |
| Function | Together with BCKDHB forms the heterotetrameric E1 subunit of the mitochondrial branched-chain alpha-ketoacid dehydrogenase (BCKD) complex. The BCKD complex catalyzes the multi-step oxidative decarboxylation of alpha-ketoacids derived from the branched-chain amino-acids valine, leucine and isoleucine producing CO2 and acyl-CoA which is subsequently utilized to produce energy. The E1 subunit catalyzes the first step with the decarboxylation of the alpha-ketoacid forming an enzyme-product intermediate. A reductive acylation mediated by the lipoylamide cofactor of E2 extracts the acyl group from the E1 active site for the next step of the reaction. |
| Protein Name | 2-Oxoisovalerate Dehydrogenase Subunit Alpha - Mitochondrial Branched-Chain Alpha-Keto Acid Dehydrogenase E1 Component Alpha Chain Bckde1a Bckdh E1-Alpha |
| Database Links | Reactome: R-HSA-70895 Reactome: R-HSA-9859138 |
| Cellular Localisation | Mitochondrion Matrix |
| Alternative Antibody Names | Anti-2-Oxoisovalerate Dehydrogenase Subunit Alpha - Mitochondrial antibody Anti-Branched-Chain Alpha-Keto Acid Dehydrogenase E1 Component Alpha Chain antibody Anti-Bckde1a antibody Anti-Bckdh E1-Alpha antibody Anti-BCKDHA antibody |
Information sourced from Uniprot.org