Reactome | ECHS1 hydrates methacrylyl-CoA

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ECHS1 hydrates methacrylyl-CoA Show undefined attributes

catalystActivity	[CatalystActivity:71049] enoyl-CoA hydratase activity of ECHS1 hexamer [mitochondrial matrix]
catalystActivityReference	[CatalystActivityReference:9672920] enoyl-CoA hydratase activity of ECHS1 hexamer [mitochondrial matrix] Clinical, biochemical and metabolic characterisation of a mild form of human short-chain enoyl-CoA hydratase deficiency: significance of increased N-acetyl-S-(2-carboxypropyl)cysteine excretion
category	transition
compartment	[Compartment:5460] mitochondrial matrix
crossReference	[DatabaseIdentifier:12667557] RHEA:31176 [DatabaseIdentifier:9917123] RHEA:31176
dbId	70870
diseaseReaction	[FailedReaction:R-HSA-9916717] ECHS1 mutants don't synthesize beta-hydroxyisobutyryl-CoA - Homo sapiens
displayName	ECHS1 hydrates methacrylyl-CoA
eventOf	[Pathway:R-HSA-70895] Branched-chain amino acid catabolism - Homo sapiens
followingEvent	[Reaction:R-HSA-70881] beta-hydroxyisobutyryl-CoA + H2O => beta-hydroxyisobutyrate + CoA - Homo sapiens
input	[SimpleEntity:R-ALL-113521] H2O [mitochondrial matrix] [SimpleEntity:R-ALL-70858] MACR-CoA [mitochondrial matrix]
isInDisease	false
isInferred	false
literatureReference	[LiteratureReference:9672910] Clinical, biochemical and metabolic characterisation of a mild form of human short-chain enoyl-CoA hydratase deficiency: significance of increased N-acetyl-S-(2-carboxypropyl)cysteine excretion
maxDepth	1
modified	[InstanceEdit:10996769] Weiser, Joel, 2026-03-16
name	ECHS1 hydrates methacrylyl-CoA (2E)-2-methylpropenoyl-CoA + H2O => (S)-3-hydroxyisobutanoyl-CoA methacrylyl-CoA + H2O <=> beta-hydroxyisobutyryl-CoA
orthologousEvent	[Reaction:R-DDI-70870] ECHS1 hydrates methacrylyl-CoA - Dictyostelium discoideum [Reaction:R-CEL-70870] ECHS1 hydrates methacrylyl-CoA - Caenorhabditis elegans [Reaction:R-DME-70870] ECHS1 hydrates methacrylyl-CoA - Drosophila melanogaster [Reaction:R-GGA-70870] ECHS1 hydrates methacrylyl-CoA - Gallus gallus [Reaction:R-XTR-70870] ECHS1 hydrates methacrylyl-CoA - Xenopus tropicalis [Reaction:R-DRE-70870] ECHS1 hydrates methacrylyl-CoA - Danio rerio [Reaction:R-SSC-70870] ECHS1 hydrates methacrylyl-CoA - Sus scrofa [Reaction:R-BTA-70870] ECHS1 hydrates methacrylyl-CoA - Bos taurus [Reaction:R-CFA-70870] ECHS1 hydrates methacrylyl-CoA - Canis familiaris [Reaction:R-RNO-70870] ECHS1 hydrates methacrylyl-CoA - Rattus norvegicus [Reaction:R-MMU-70870] ECHS1 hydrates methacrylyl-CoA - Mus musculus
output	[SimpleEntity:R-ALL-71847] bHIB-CoA [mitochondrial matrix]
precedingEvent	[Reaction:R-HSA-70859] isobutyryl-CoA + FAD => methacrylyl-CoA + FADH2 - Homo sapiens
releaseDate	2004-07-06
reviewStatus	[ReviewStatus:9821382] five stars
reviewed	[InstanceEdit:9672923] Jassal, Bijay, 2020-01-03
revised	[InstanceEdit:9672777] D'Eustachio, Peter, 2020-01-02
schemaClass	Reaction
species	[Species:48887] Homo sapiens
stId	R-HSA-70870
summation	[Summation:178949] Mitochondrial ECHS1 (enoyl-CoA hydratase) hexamer catalyzes ...
updateTrackers	[UpdateTracker:9777105] Update Tracker - [Reaction:70870] ECHS1 hydrates methacrylyl-CoA - v72:[add_removeCatalystActivity, add_removeLiteratureReference, modifyText]

Referrals

(updatedInstance)	[UpdateTracker:9777105] Update Tracker - [Reaction:70870] ECHS1 hydrates methacrylyl-CoA - v72:[add_removeCatalystActivity, add_removeLiteratureReference, modifyText]
(normalReaction)	[FailedReaction:R-HSA-9916717] ECHS1 mutants don't synthesize beta-hydroxyisobutyryl-CoA
(hasEvent)	[Pathway:R-HSA-70895] Branched-chain amino acid catabolism
(precedingEvent)	[Reaction:R-HSA-70881] beta-hydroxyisobutyryl-CoA + H2O => beta-hydroxyisobutyrate + CoA