| Function | Short and branched chain specific acyl-CoA dehydrogenase that catalyzes the removal of one hydrogen from C-2 and C-3 of the fatty acyl-CoA thioester, resulting in the formation of trans-2-enoyl-CoA. Among the different mitochondrial acyl-CoA dehydrogenases, acts specifically on short and branched chain acyl-CoA derivatives such as (S)-2-methylbutyryl-CoA as well as short straight chain acyl-CoAs such as butyryl-CoA. Plays an important role in the metabolism of L-isoleucine by catalyzing the dehydrogenation of 2-methylbutyryl-CoA, one of the steps of the L-isoleucine catabolic pathway. Can also act on valproyl-CoA, a metabolite of valproic acid, an antiepileptic drug. |
| Protein Name | Short/Branched Chain Specific Acyl-Coa Dehydrogenase - MitochondrialSbcad2-Methyl Branched Chain Acyl-Coa Dehydrogenase2-Mebcad2-Methylbutyryl-Coenzyme A Dehydrogenase2-Methylbutyryl-Coa Dehydrogenase |
| Database Links | Reactome: R-HSA-70895Reactome: R-HSA-9837999 |
| Cellular Localisation | Mitochondrion Matrix |
| Alternative Protein Names | Short/Branched Chain Specific Acyl-Coa Dehydrogenase - Mitochondrial proteinSbcad protein2-Methyl Branched Chain Acyl-Coa Dehydrogenase protein2-Mebcad protein2-Methylbutyryl-Coenzyme A Dehydrogenase protein2-Methylbutyryl-Coa Dehydrogenase proteinACADSB protein |
Information sourced from Uniprot.org
