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Details on Person UniProt:P45954 ACADSB
| Class:Id | ReferenceGeneProduct:49488 |
|---|---|
| _chainChangeLog | transit peptide:1-33 added on Sat February 7 2015;chain:34-432 added on Sat February 7 2015 |
| _displayName | UniProt:P45954 ACADSB |
| _timestamp | 2026-02-20 21:36:43 |
| chain | transit peptide:1-33 chain:34-432 |
| checksum | 1EB5F894B1944E99 |
| comment | FUNCTION Short and branched chain specific acyl-CoA dehydrogenase that catalyzes the proR-proR stereospecific alpha,beta-dehydrogenation of fatty acyl-CoA thioesters using the electron transfer flavoprotein (ETF) as their physiologic electron acceptor, resulting in the formation of trans-2-enoyl-CoA ((2E)-enoyl-CoA) (PubMed:10832746, PubMed:11013134, PubMed:21430231, PubMed:7698750). 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 (butanoyl-CoA) (PubMed:10832746, PubMed:11013134, PubMed:21430231, PubMed:7698750). 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 (PubMed:10832746, PubMed:11013134). Can also act on valproyl-CoA, a metabolite of valproic acid, an antiepileptic drug (PubMed:8660691).CATALYTIC ACTIVITY a short-chain 2,3-saturated fatty acyl-CoA + oxidized [electron-transfer flavoprotein] + H(+) = a short-chain (2E)-enoyl-CoA + reduced [electron-transfer flavoprotein]CATALYTIC ACTIVITY 2-methylbutanoyl-CoA + oxidized [electron-transfer flavoprotein] + H(+) = (2E)-2-methylbut-2-enoyl-CoA + reduced [electron-transfer flavoprotein]CATALYTIC ACTIVITY (2S)-2-methylbutanoyl-CoA + oxidized [electron-transfer flavoprotein] + H(+) = (2E)-2-methylbut-2-enoyl-CoA + reduced [electron-transfer flavoprotein]CATALYTIC ACTIVITY (2R)-2-methylbutanoyl-CoA + oxidized [electron-transfer flavoprotein] + H(+) = ethylacryloyl-CoA + reduced [electron-transfer flavoprotein]CATALYTIC ACTIVITY 2-methylpropanoyl-CoA + oxidized [electron-transfer flavoprotein] + H(+) = 2-methylpropenoyl-CoA + reduced [electron-transfer flavoprotein]CATALYTIC ACTIVITY 2-methylhexanoyl-CoA + oxidized [electron-transfer flavoprotein] + H(+) = 2-methylhexenoyl-CoA + reduced [electron-transfer flavoprotein]CATALYTIC ACTIVITY valproyl-CoA + oxidized [electron-transfer flavoprotein] + H(+) = (2E)-2-propylpent-2-enoyl-CoA + reduced [electron-transfer flavoprotein]CATALYTIC ACTIVITY butanoyl-CoA + oxidized [electron-transfer flavoprotein] + H(+) = (2E)-butenoyl-CoA + reduced [electron-transfer flavoprotein]CATALYTIC ACTIVITY hexanoyl-CoA + oxidized [electron-transfer flavoprotein] + H(+) = (2E)-hexenoyl-CoA + reduced [electron-transfer flavoprotein]COFACTOR Competitively inhibited by valproyl-CoA.BIOPHYSICOCHEMICAL PROPERTIES kcat is 9700 sec(-1) for the dehydrogenation of (2S)-2-methylbutanoyl-CoA (PubMed:12855692). kcat is 7600 sec(-1) for the dehydrogenation of hexanoyl-CoA (PubMed:12855692). kcat is 2900 sec(-1) for the dehydrogenation of hexanoyl-CoA (PubMed:12855692).PATHWAY Lipid metabolism; mitochondrial fatty acid beta-oxidation.PATHWAY Amino-acid degradation; L-isoleucine degradation.SUBUNIT Homotetramer.SUBCELLULAR LOCATION Ubiquitously expressed, with highest levels in heart, muscle and liver.DISEASE The disease is caused by variants affecting the gene represented in this entry.SIMILARITY Belongs to the acyl-CoA dehydrogenase family. |
| description | recommendedName: fullName evidence="19"Short/branched chain specific acyl-CoA dehydrogenase, mitochondrial shortName evidence="16"SBCAD ecNumber evidence="5 12"1.3.8.1 alternativeName: 2-methyl branched chain acyl-CoA dehydrogenase shortName: 2-MEBCAD alternativeName: 2-methylbutyryl-coenzyme A dehydrogenase shortName: 2-methylbutyryl-CoA dehydrogenase ecNumber evidence="3 11"1.3.8.5 |
| geneName | ACADSB |
| identifier | P45954 |
| isSequenceChanged | FALSE |
| keyword | 3D-structure Acetylation Alternative splicing Disease variant FAD Fatty acid metabolism Flavoprotein Lipid metabolism Mitochondrion Oxidoreductase Phosphoprotein Proteomics identification Reference proteome Transit peptide |
| modified | [InstanceEdit:9836292] Weiser, Joel, 2023-05-25 [InstanceEdit:9852000] Weiser, Joel, 2023-11-03 [InstanceEdit:9917590] Weiser, Joel, 2024-08-09 [InstanceEdit:9926675] Weiser, Joel, 2024-11-03 [InstanceEdit:9939033] Weiser, Joel, 2025-02-21 [InstanceEdit:9948485] Weiser, Joel, 2025-05-21 [InstanceEdit:9983091] Weiser, Joel, 2026-02-20 |
| name | ACADSB |
| referenceDatabase | [ReferenceDatabase:2] UniProt |
| referenceGene | [ReferenceDNASequence:8995827] ENSEMBL:ENSG00000196177 ACADSB [Homo sapiens] |
| secondaryIdentifier | ACDSB_HUMAN B4DQ51 Q5SQN6 Q96CX7 |
| sequenceLength | 432 |
| species | [Species:48887] Homo sapiens |
| (isoformParent) | [ReferenceIsoform:8972686] UniProt:P45954-1 ACADSB [Homo sapiens] [ReferenceIsoform:8972687] UniProt:P45954-2 ACADSB [Homo sapiens] |
| (referenceEntity) | [EntityWithAccessionedSequence:70796] ACADSB(52-432) [mitochondrial matrix] [Homo sapiens] |
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No pathways have been reviewed or authored by UniProt:P45954 ACADSB (49488)
