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Proteins/UNIPROT:P45954
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). 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). 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..
Function from UniProtKB (P45954), read 2026-09-23. CC BY 4.0.
Curated compounds routed: 0
1 row
59 statements
59 statements · 8 reference databases · Subject: ACADSB · Sources: Bgee, Hetionet, Human Protein Atlas, Human-GEM, Reactome, Rhea, UniProt, WikiPathways
16 reactions
FAD+CHEBI:27540(2E)-hexenoyl-CoA+FADH2
Catalysed by ACOX3 or ACOX1 or ACADSB
Reaction pageLeft to rightBalancedEC 1.3.8.5, 1.3.3.6mitochondrial matrix
Human-GEM MAR03156CC BY 4.0Reference data
5 names
Resolves to: ACADSB
Reaction pageLeft to rightBalancedEC 1.3.8.7mitochondrial matrix
Human-GEM MAR03163CC BY 4.0Reference data
Catalysed by ACADSB
Reaction pageLeft to rightBalancedEC 1.3.8.5mitochondrial matrix
Human-GEM MAR03202CC BY 4.0Reference data
FAD+propionyl-CoAacryloyl-CoA+FADH2
Reaction pageLeft to rightBalancedEC 1.3.8.7, 1.3.8.8, 1.3.8.9mitochondrial matrix
Human-GEM MAR03212CC BY 4.0Reference data
FADH2+MAM02468mFAD+isobutyryl-CoA
Catalysed by ACAD8 or ACADS or ACADSB
Reaction pageReversibleBalancedEC 1.3.8.5, 1.3.8.1mitochondrial matrix
Human-GEM MAR03752CC BY 4.0Reference data
3-methylbut-2-enoyl-CoA+FADH2FAD+isovaleryl-CoA
Reaction pageReversibleBalancedEC 1.3.8.4, 1.3.8.5mitochondrial matrix
Human-GEM MAR03770CC BY 4.0Reference data
CHEBI:15477+FADFADH2+2-methylcrotonoyl-CoA
Catalysed by ACAD8 or ACADS or ACADSB
Reaction pageReversibleBalancedEC 1.3.8.5, 1.3.8.1mitochondrial matrix
Human-GEM MAR03784CC BY 4.0Reference data
butanoyl-CoA+oxidized [electron-transfer flavoprotein]+hydron(2E)-butenoyl-CoA+reduced [electron-transfer flavoprotein]
Catalysed by ACADS or IVD or ACADSB
Reaction pageLeft to rightBalance undeterminable
Rhea RHEA:24004CC BY 4.0Reference data
hexanoyl-CoA+oxidized [electron-transfer flavoprotein]+hydron(2E)-hexenoyl-CoA+reduced [electron-transfer flavoprotein]
Catalysed by ACADM or ACADS or IVD or ACADL or ACADSB
Reaction pageLeft to rightBalance undeterminable
Rhea RHEA:43464CC BY 4.0Reference data
2-methylbutanoyl-CoA+oxidized [electron-transfer flavoprotein]+hydron2-methylcrotonoyl-CoA+reduced [electron-transfer flavoprotein]
Catalysed by ACADSB
Reaction pageLeft to rightBalance undeterminable
Rhea RHEA:43780CC BY 4.0Reference data
2-methylpropanoyl-CoA+oxidized [electron-transfer flavoprotein]+hydronCHEBI:62500+reduced [electron-transfer flavoprotein]
Reaction pageLeft to rightBalance undeterminable
Rhea RHEA:44180CC BY 4.0Reference data
CHEBI:87487+oxidized [electron-transfer flavoprotein]+hydronCHEBI:87488+reduced [electron-transfer flavoprotein]
Catalysed by ACADS or IVD or ACADSB
Reaction pageLeft to rightBalance undeterminable
Rhea RHEA:47196CC BY 4.0Reference data