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Proteins/UNIPROT:P26440
A mitochondrial matrix enzyme that catalyzes the third step in leucine catabolism, where isovaleryl-CoA (3-methylbutanoyl-CoA) is metabolized to 3-methylbut-2-enoyl-CoA. To a lesser extent, it also participates in the first step in fatty acid beta-oxidation, in which it catalyzes the proR-proR stereospecific alpha,beta-dehydrogenation of other saturated short-chain acyl-CoA thioesters such as pentanoyl-CoA, hexanoyl-CoA and butanoyl-CoA, using the electron transfer flavoprotein (ETF) as their physiologic electron acceptor..
Function from UniProtKB (P26440), read 2026-09-23. CC BY 4.0.
Curated compounds routed: 0
1 row
75 statements
75 statements · 7 reference databases · Subject: IVD · Sources: Bgee, Human Protein Atlas, Human-GEM, Reactome, Rhea, UniProt, WikiPathways
6 reactions
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
isovaleryl-CoA+oxidized [electron-transfer flavoprotein]+hydron3-methylbut-2-enoyl-CoA+reduced [electron-transfer flavoprotein]
Catalysed by IVD
1 name
Resolves to: IVD
Rhea RHEA:12276CC 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
CHEBI:57389+oxidized [electron-transfer flavoprotein]+hydronCHEBI:86160+reduced [electron-transfer flavoprotein]
Catalysed by ACADM or ACADS or IVD
Reaction pageLeft to rightBalance undeterminable
Rhea RHEA:43456CC 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
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