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Schema
>
Reaction
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Entries
isovaleryl-CoA + FAD => beta-methylcrotonyl-CoA + FADH2
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catalystActivity
[CatalystActivity:70743] 3-methylbutanoyl-CoA dehydrogenase activity of IVD tetramer [mitochondrial matrix]
catalystActivityReference
[CatalystActivityReference:9644049] isovaleryl-CoA dehydrogenase activity of IVD tetramer [mitochondrial matrix] Purification and properties of short chain acyl-CoA, medium chain acyl-CoA, and isovaleryl-CoA dehydrogenases from human liver
category
transition
compartment
[Compartment:5460] mitochondrial matrix
crossReference
[DatabaseIdentifier:9917119] RHEA:12277
dbId
70745
displayName
isovaleryl-CoA + FAD => beta-methylcrotonyl-CoA + FADH2
eventOf
[Pathway:R-HSA-70895] Branched-chain amino acid catabolism - Homo sapiens
followingEvent
[Reaction:R-HSA-70773] beta-methylcrotonyl-CoA + ATP + HCO3- <=> beta-methylglutaconyl-CoA + ADP + orthophosphate + H2O (MCCA) - Homo sapiens
input
[SimpleEntity:R-ALL-113596] FAD [mitochondrial matrix]
[SimpleEntity:R-ALL-113529] H+ [mitochondrial matrix]
[SimpleEntity:R-ALL-70715] ISV-CoA [mitochondrial matrix]
isInDisease
false
isInferred
false
literatureReference
[LiteratureReference:70739] Purification and properties of short chain acyl-CoA, medium chain acyl-CoA, and isovaleryl-CoA dehydrogenases from human liver
[LiteratureReference:70746] Demonstration of a specific mitochondrial isovaleryl-CoA dehydrogenase deficiency in fibroblasts from patients with isovaleric acidemia
[LiteratureReference:70731] Structure of human isovaleryl-CoA dehydrogenase at 2.6 A resolution: structural basis for substrate specificity
maxDepth
1
modified
[InstanceEdit:10948619] Weiser, Joel, 2025年08月27日
name
isovaleryl-CoA + FAD => beta-methylcrotonyl-CoA + FADH2
3-methylbutanoyl-CoA + H(+) + oxidized (electron-transfer flavoprotein) => 3-methyl-(2E)-butenoyl-CoA + reduced (electron-transfer flavoprotein)
orthologousEvent
[Reaction:R-MMU-70745] isovaleryl-CoA + FAD => beta-methylcrotonyl-CoA + FADH2 - Mus musculus
[Reaction:R-DDI-70745] isovaleryl-CoA + FAD => beta-methylcrotonyl-CoA + FADH2 - Dictyostelium discoideum
[Reaction:R-CEL-70745] isovaleryl-CoA + FAD => beta-methylcrotonyl-CoA + FADH2 - Caenorhabditis elegans
[Reaction:R-DME-70745] isovaleryl-CoA + FAD => beta-methylcrotonyl-CoA + FADH2 - Drosophila melanogaster
[Reaction:R-GGA-70745] isovaleryl-CoA + FAD => beta-methylcrotonyl-CoA + FADH2 - Gallus gallus
[Reaction:R-XTR-70745] isovaleryl-CoA + FAD => beta-methylcrotonyl-CoA + FADH2 - Xenopus tropicalis
[Reaction:R-DRE-70745] isovaleryl-CoA + FAD => beta-methylcrotonyl-CoA + FADH2 - Danio rerio
[Reaction:R-SSC-70745] isovaleryl-CoA + FAD => beta-methylcrotonyl-CoA + FADH2 - Sus scrofa
[Reaction:R-BTA-70745] isovaleryl-CoA + FAD => beta-methylcrotonyl-CoA + FADH2 - Bos taurus
[Reaction:R-CFA-70745] isovaleryl-CoA + FAD => beta-methylcrotonyl-CoA + FADH2 - Canis familiaris
[Reaction:R-RNO-70745] isovaleryl-CoA + FAD => beta-methylcrotonyl-CoA + FADH2 - Rattus norvegicus
output
[SimpleEntity:R-ALL-70744] bMC-CoA [mitochondrial matrix]
[SimpleEntity:R-ALL-31649] FADH2 [mitochondrial matrix]
precedingEvent
[Reaction:R-HSA-9859163] DBT transfers BCAA to CoA - Homo sapiens
previousReviewStatus
[ReviewStatus:9821385] four stars
releaseDate
2004年07月06日
reviewStatus
[ReviewStatus:9821382] five stars
reviewed
[InstanceEdit:9919316] D'Eustachio, Peter, 2024年08月21日
schemaClass
Reaction
species
[Species:48887] Homo sapiens
stId
R-HSA-70745
summation
[Summation:178922] Mitochondrial isovaleryl dehydrogenase (IVD) catalyzes the r...
updateTrackers
[UpdateTracker:9791165] Update Tracker - [Reaction:70745] isovaleryl-CoA + FAD => beta-methylcrotonyl-CoA + FADH2 - v32:[addLiteratureReference, modifyText]
[UpdateTracker:9919378] Update Tracker - [Reaction:70745] isovaleryl-CoA + FAD => beta-methylcrotonyl-CoA + FADH2 - v90:[modifyText, removeInput]
Referrals
(updatedInstance)
[UpdateTracker:9791165] Update Tracker - [Reaction:70745] isovaleryl-CoA + FAD => beta-methylcrotonyl-CoA + FADH2 - v32:[addLiteratureReference, modifyText]
[UpdateTracker:9919378] Update Tracker - [Reaction:70745] isovaleryl-CoA + FAD => beta-methylcrotonyl-CoA + FADH2 - v90:[modifyText, removeInput]
(normalReaction)
[FailedReaction:R-HSA-9914837] IVD mutants don't synthesize beta-methylcrotonyl-CoA
(hasEvent)
[Pathway:R-HSA-70895] Branched-chain amino acid catabolism
(precedingEvent)
[Reaction:R-HSA-70773] beta-methylcrotonyl-CoA + ATP + HCO3- <=> beta-methylglutaconyl-CoA + ADP + orthophosphate + H2O (MCCA)
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