| Host: |
Rabbit |
| Applications: |
WB/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 0.01% Thimerosal, 50% Glycerol, pH 7.3. |
| Purification: |
Affinity purification |
| Concentration: |
Lot specific |
| Dilution Range: |
WB:1:500-1:2000ELISA:Recommended starting concentration is 1 Mu g/mL. Please optimize the concentration based on your specific assay requirements. |
| Gene Symbol: |
BCKDHA |
| Gene ID: |
593 |
| Uniprot ID: |
ODBA_HUMAN |
| Immunogen Region: |
276-445 aa |
| 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 - MitochondrialBranched-Chain Alpha-Keto Acid Dehydrogenase E1 Component Alpha ChainBckde1aBckdh E1-Alpha |
| Database Links | Reactome: R-HSA-70895Reactome: R-HSA-9859138 |
| Cellular Localisation | Mitochondrion Matrix |
| Alternative Antibody Names | Anti-2-Oxoisovalerate Dehydrogenase Subunit Alpha - Mitochondrial antibodyAnti-Branched-Chain Alpha-Keto Acid Dehydrogenase E1 Component Alpha Chain antibodyAnti-Bckde1a antibodyAnti-Bckdh E1-Alpha antibodyAnti-BCKDHA antibody |
Information sourced from Uniprot.org
12 months for antibodies. 6 months for ELISA Kits. Please see website T&Cs for further guidance
