| Function | Together with BCKDHA 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 Beta - MitochondrialBranched-Chain Alpha-Keto Acid Dehydrogenase E1 Component Beta ChainBckde1bBckdh E1-Beta |
| Database Links | Reactome: R-HSA-70895Reactome: R-HSA-9859138Reactome: R-HSA-9865113Reactome: R-HSA-9865125Reactome: R-HSA-9907570Reactome: R-HSA-9912481Reactome: R-HSA-9912529 |
| Cellular Localisation | Mitochondrion Matrix |
| Alternative Antibody Names | Anti-2-Oxoisovalerate Dehydrogenase Subunit Beta - Mitochondrial antibodyAnti-Branched-Chain Alpha-Keto Acid Dehydrogenase E1 Component Beta Chain antibodyAnti-Bckde1b antibodyAnti-Bckdh E1-Beta antibodyAnti-BCKDHB antibody |
Information sourced from Uniprot.org
