| 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
