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Details on Person PAH:Fe2+ tetramer hydroxylates L-Phe to L-Tyr
Class:Id Reaction:71118
_displayName PAH:Fe2+ tetramer hydroxylates L-Phe to L-Tyr
_doRelease TRUE
_timestamp 2024-12-03 20:52:48
authored [InstanceEdit:73677] D'Eustachio, P, 2003-06-24 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
compartment [Compartment:70101] cytosol
crossReference [DatabaseIdentifier:9929215] RHEA:20274
edited [InstanceEdit:73677] D'Eustachio, P, 2003-06-24 00:00:00
input [SimpleEntity:29502] L-Phe [cytosol] [SimpleEntity:29870] BH4 [cytosol] [SimpleEntity:29368] O2 [cytosol]
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
modified [InstanceEdit:163376] Vastrik, I, 2005-04-29 06:26:51 [InstanceEdit:517476] D'Eustachio, P, 2010-02-18 [InstanceEdit:584171] D'Eustachio, P, 2010-04-11 [InstanceEdit:1080814] D'Eustachio, P, 2010-11-27 [InstanceEdit:2247552] D'Eustachio, P, 2012-05-14 [InstanceEdit:5649482] Jassal, Bijay, 2014-11-20 [InstanceEdit:5663028] D'Eustachio, Peter, 2015-01-14 [InstanceEdit:8946993] Shorser, Solomon, 2016-11-07 [InstanceEdit:9643047] Wu, G, 2019-04-04 [InstanceEdit:9644799] Jassal, Bijay, 2019-04-24 [InstanceEdit:9830342] Matthews, Lisa, 2023-03-08 [InstanceEdit:9929230] D'Eustachio, Peter, 2024-11-22 [InstanceEdit:9930378] D'Eustachio, Peter, 2024-12-03
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
output [SimpleEntity:29506] L-Tyr [cytosol] [SimpleEntity:71112] 4aOH-BH4 [cytosol]
precedingEvent
releaseDate 2004-07-06
reviewStatus [ReviewStatus:9821382] five stars
species [Species:48887] Homo sapiens
stableIdentifier [StableIdentifier:361423] R-HSA-71118.5
summation [Summation:179034] Cytosolic phenylalanine hydroxylase (PAH) catalyzes the reac...
(hasEvent) [Pathway:8964208] Phenylalanine metabolism [Homo sapiens]
(normalReaction) [FailedReaction:5649483] Defective PAH does not hydroxylate L-Phe to L-Tyr [Homo sapiens]
(precedingEvent) [Reaction:71146] 4a-hydroxytetrahydrobiopterin => q-dihydrobiopterin + H2O [Homo sapiens] [Reaction:71155] TAT deaminates tyrosine [Homo sapiens]
(updatedInstance) [_UpdateTracker:9780411] Update Tracker - [Reaction:71118] PAH:Fe2+ tetramer hydroxylates L-Phe to L-Tyr - v63:[modifyText] [_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:9790778] Update Tracker - [Reaction:71118] PAH:Fe2+ tetramer hydroxylates L-Phe to L-Tyr - v33:[removeOutput] [_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]
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