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Entries
PAH:Fe2+ tetramer hydroxylates L-Phe to L-Tyr
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authored
[InstanceEdit:73677] D'Eustachio, P, 2003年06月24日 00:00:00
catalystActivity
[CatalystActivity:71074] phenylalanine 4-monooxygenase activity of PAH:Fe2+ tetramer [cytosol]
catalystActivityReference
[CatalystActivityReference:9643292] phenylalanine 4-monooxygenase activity of PAH:Fe2+ tetramer [cytosol] Structure of tetrameric human phenylalanine hydroxylase and its implications for phenylketonuria
category
transition
compartment
[Compartment:70101] cytosol
crossReference
[DatabaseIdentifier:9929215] RHEA:20274
dbId
71118
displayName
PAH:Fe2+ tetramer hydroxylates L-Phe to L-Tyr
edited
[InstanceEdit:73677] D'Eustachio, P, 2003年06月24日 00:00:00
eventOf
[Pathway:R-HSA-8964208] Phenylalanine metabolism - Homo sapiens
followingEvent
[Reaction:R-HSA-71155] TAT deaminates tyrosine - Homo sapiens
[Reaction:R-HSA-71146] 4a-hydroxytetrahydrobiopterin => q-dihydrobiopterin + H2O - Homo sapiens
input
[SimpleEntity:R-ALL-29870] BH4 [cytosol]
[SimpleEntity:R-ALL-29502] L-Phe [cytosol]
[SimpleEntity:R-ALL-29368] O2 [cytosol]
isInDisease
false
isInferred
false
literatureReference
[LiteratureReference:9929222] Crystal structure of the ternary complex of the catalytic domain of human phenylalanine hydroxylase with tetrahydrobiopterin and 3-(2-thienyl)-L-alanine, and its implications for the mechanism of catalysis and substrate activation
[LiteratureReference:71069] Structure of tetrameric human phenylalanine hydroxylase and its implications for phenylketonuria
[LiteratureReference:2160457] Phenylalanine hydroxylase deficiency in a population in Germany: mutational profile and nine novel mutations
maxDepth
1
modified
[InstanceEdit:10948619] Weiser, Joel, 2025年08月27日
name
PAH:Fe2+ tetramer hydroxylates L-Phe to L-Tyr
(6R)-L-erythro-5,6,7,8-tetrahydrobiopterin + L-phenylalanine + O2 => (4aS,6R)-4a-hydroxy-L-erythro-5,6,7,8-tetrahydrobiopterin + L-tyrosine
phenylalanine + tetrahydrobiopterin + O2 => tyrosine + 4a-hydroxytetrahydrobiopterin + H2O
orthologousEvent
[Reaction:R-DDI-71118] PAH:Fe2+ tetramer hydroxylates L-Phe to L-Tyr - Dictyostelium discoideum
[Reaction:R-CEL-71118] PAH:Fe2+ tetramer hydroxylates L-Phe to L-Tyr - Caenorhabditis elegans
[Reaction:R-DME-71118] PAH:Fe2+ tetramer hydroxylates L-Phe to L-Tyr - Drosophila melanogaster
[Reaction:R-GGA-71118] PAH:Fe2+ tetramer hydroxylates L-Phe to L-Tyr - Gallus gallus
[Reaction:R-XTR-71118] PAH:Fe2+ tetramer hydroxylates L-Phe to L-Tyr - Xenopus tropicalis
[Reaction:R-DRE-71118] PAH:Fe2+ tetramer hydroxylates L-Phe to L-Tyr - Danio rerio
[Reaction:R-SSC-71118] PAH:Fe2+ tetramer hydroxylates L-Phe to L-Tyr - Sus scrofa
[Reaction:R-BTA-71118] PAH:Fe2+ tetramer hydroxylates L-Phe to L-Tyr - Bos taurus
[Reaction:R-CFA-71118] PAH:Fe2+ tetramer hydroxylates L-Phe to L-Tyr - Canis familiaris
[Reaction:R-RNO-71118] PAH:Fe2+ tetramer hydroxylates L-Phe to L-Tyr - Rattus norvegicus
[Reaction:R-MMU-71118] PAH:Fe2+ tetramer hydroxylates L-Phe to L-Tyr - Mus musculus
output
[SimpleEntity:R-ALL-71112] 4aOH-BH4 [cytosol]
[SimpleEntity:R-ALL-29506] L-Tyr [cytosol]
precedingEvent
[Reaction:R-HSA-71130] q-dihydrobiopterin + NADH + H+ => tetrahydrobiopterin + NAD+ - Homo sapiens
[Reaction:R-HSA-5696365] ASRGL1 hydrolyses aspartame to L-Asp, L-Phe, MeOH - Homo sapiens
releaseDate
2004年07月06日
reviewStatus
[ReviewStatus:9821382] five stars
schemaClass
Reaction
species
[Species:48887] Homo sapiens
stId
R-HSA-71118
summation
[Summation:179034] Cytosolic phenylalanine hydroxylase (PAH) catalyzes the reac...
updateTrackers
[UpdateTracker:9785366] Update Tracker - [Reaction:71118] PAH:Fe2+ tetramer hydroxylates L-Phe to L-Tyr - v52:[addLiteratureReference, modifyText, removeRegulator]
[UpdateTracker:9788688] Update Tracker - [Reaction:71118] PAH:Fe2+ tetramer hydroxylates L-Phe to L-Tyr - v41:[addRegulator]
[UpdateTracker:9791133] Update Tracker - [Reaction:71118] PAH:Fe2+ tetramer hydroxylates L-Phe to L-Tyr - v32:[modifyText]
[UpdateTracker:9939635] Update Tracker - [Reaction:71118] PAH:Fe2+ tetramer hydroxylates L-Phe to L-Tyr - v92:[add_removeLiteratureReference, modifyText]
[UpdateTracker:9780411] Update Tracker - [Reaction:71118] PAH:Fe2+ tetramer hydroxylates L-Phe to L-Tyr - v63:[modifyText]
[UpdateTracker:9790778] Update Tracker - [Reaction:71118] PAH:Fe2+ tetramer hydroxylates L-Phe to L-Tyr - v33:[removeOutput]
Referrals
(updatedInstance)
[UpdateTracker:9785366] Update Tracker - [Reaction:71118] PAH:Fe2+ tetramer hydroxylates L-Phe to L-Tyr - v52:[addLiteratureReference, modifyText, removeRegulator]
[UpdateTracker:9788688] Update Tracker - [Reaction:71118] PAH:Fe2+ tetramer hydroxylates L-Phe to L-Tyr - v41:[addRegulator]
[UpdateTracker:9791133] Update Tracker - [Reaction:71118] PAH:Fe2+ tetramer hydroxylates L-Phe to L-Tyr - v32:[modifyText]
[UpdateTracker:9939635] Update Tracker - [Reaction:71118] PAH:Fe2+ tetramer hydroxylates L-Phe to L-Tyr - v92:[add_removeLiteratureReference, modifyText]
[UpdateTracker:9780411] Update Tracker - [Reaction:71118] PAH:Fe2+ tetramer hydroxylates L-Phe to L-Tyr - v63:[modifyText]
[UpdateTracker:9790778] Update Tracker - [Reaction:71118] PAH:Fe2+ tetramer hydroxylates L-Phe to L-Tyr - v33:[removeOutput]
(normalReaction)
[FailedReaction:R-HSA-5649483] Defective PAH does not hydroxylate L-Phe to L-Tyr
(hasEvent)
[Pathway:R-HSA-8964208] Phenylalanine metabolism
(precedingEvent)
[Reaction:R-HSA-71155] TAT deaminates tyrosine
[Reaction:R-HSA-71146] 4a-hydroxytetrahydrobiopterin => q-dihydrobiopterin + H2O
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