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Schema
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Entries
FH tetramer hydrates fumarate to L-malate
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catalystActivity
[CatalystActivity:70981] fumarate hydratase activity of FH tetramer [mitochondrial matrix]
catalystActivityReference
[CatalystActivityReference:9643531] fumarate hydratase activity of FH tetramer [mitochondrial matrix] Mutation of the fumarase gene in two siblings with progressive encephalopathy and fumarase deficiency
category
transition
compartment
[Compartment:5460] mitochondrial matrix
crossReference
[DatabaseIdentifier:12667547] RHEA:12462
[DatabaseIdentifier:9854203] RHEA:12460
dbId
70982
displayName
FH tetramer hydrates fumarate to L-malate
eventOf
[Pathway:R-HSA-71403] Citric acid cycle (TCA cycle) - Homo sapiens
followingEvent
[Reaction:R-HSA-70979] MDH2 dimer dehydrogenates MAL - Homo sapiens
[Reaction:R-HSA-372843] SLC25A10 mediates exchange of malate and phosphate - Homo sapiens
[Reaction:R-HSA-9012349] ME3 tetramer decarboxylates MAL to PYR - Homo sapiens
[Reaction:R-HSA-9012268] ME2 tetramer decarboxylates MAL to PYR - Homo sapiens
input
[SimpleEntity:R-ALL-113588] FUMA [mitochondrial matrix]
[SimpleEntity:R-ALL-113521] H2O [mitochondrial matrix]
isInDisease
false
isInferred
false
literatureReference
[LiteratureReference:198390] Mutation of the fumarase gene in two siblings with progressive encephalopathy and fumarase deficiency
[LiteratureReference:9854194] Structural, biochemical and biophysical characterization of recombinant human fumarate hydratase
[LiteratureReference:9854210] The Pleiotropic Effects of Fumarate: From Mitochondrial Respiration to Epigenetic Rewiring and DNA Repair Mechanisms
[LiteratureReference:9854207] Post-translational Modifications of Fumarase Regulate its Enzyme Activity and Function in Respiration and the DNA Damage Response
maxDepth
1
modified
[InstanceEdit:11080360] Weiser, Joel, 2026-03-16
name
FH tetramer hydrates fumarate to L-malate
fumarate + H2O => (S)-malate
orthologousEvent
[Reaction:R-GGA-373145] fumarate + H2O <=> malate - Gallus gallus
[Reaction:R-MMU-70982] FH tetramer hydrates fumarate to L-malate - Mus musculus
[Reaction:R-RNO-70982] FH tetramer hydrates fumarate to L-malate - Rattus norvegicus
[Reaction:R-CFA-70982] FH tetramer hydrates fumarate to L-malate - Canis familiaris
[Reaction:R-BTA-70982] FH tetramer hydrates fumarate to L-malate - Bos taurus
[Reaction:R-DRE-70982] FH tetramer hydrates fumarate to L-malate - Danio rerio
[Reaction:R-DDI-70982] FH tetramer hydrates fumarate to L-malate - Dictyostelium discoideum
[Reaction:R-DME-70982] FH tetramer hydrates fumarate to L-malate - Drosophila melanogaster
[Reaction:R-SCE-70982] FH tetramer hydrates fumarate to L-malate - Saccharomyces cerevisiae
[Reaction:R-SPO-70982] FH tetramer hydrates fumarate to L-malate - Schizosaccharomyces pombe
[Reaction:R-GGA-70982] FH tetramer hydrates fumarate to L-malate - Gallus gallus
[Reaction:R-CEL-70982] FH tetramer hydrates fumarate to L-malate - Caenorhabditis elegans
output
[SimpleEntity:R-ALL-113544] MAL [mitochondrial matrix]
precedingEvent
[Reaction:R-HSA-70994] SDH complex dehydrogenates succinate - Homo sapiens
releaseDate
2004-07-06
reviewStatus
[ReviewStatus:9821382] five stars
reviewed
[InstanceEdit:9861215] Hill, David P, 2024-02-15
revised
[InstanceEdit:451045] D'Eustachio, P, 2009-12-26
schemaClass
Reaction
species
[Species:48887] Homo sapiens
stId
R-HSA-70982
summation
[Summation:71412] Mitochondrial fumarate hydratase (FH) tetramer catalyzes the...
updateTrackers
[UpdateTracker:9864254] Update Tracker - [Reaction:70982] FH tetramer hydrates fumarate to L-malate - v88:[addLiteratureReference, modifyText]
[UpdateTracker:9792210] Update Tracker - [Reaction:70982] Fumarate + H2O <=> (S)-Malate - v22:[addLiteratureReference, modifyText]
[UpdateTracker:9790955] Update Tracker - [Reaction:70982] Fumarate + H2O <=> (S)-Malate - v32:[modifyText]
Referrals
(updatedInstance)
[UpdateTracker:9864254] Update Tracker - [Reaction:70982] FH tetramer hydrates fumarate to L-malate - v88:[addLiteratureReference, modifyText]
[UpdateTracker:9792210] Update Tracker - [Reaction:70982] Fumarate + H2O <=> (S)-Malate - v22:[addLiteratureReference, modifyText]
[UpdateTracker:9790955] Update Tracker - [Reaction:70982] Fumarate + H2O <=> (S)-Malate - v32:[modifyText]
(hasEvent)
[Pathway:R-HSA-71403] Citric acid cycle (TCA cycle)
(precedingEvent)
[Reaction:R-HSA-70979] MDH2 dimer dehydrogenates MAL
[Reaction:R-HSA-372843] SLC25A10 mediates exchange of malate and phosphate
[Reaction:R-HSA-9012349] ME3 tetramer decarboxylates MAL to PYR
[Reaction:R-HSA-9012268] ME2 tetramer decarboxylates MAL to PYR
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