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Details on Person Defective PAH does not hydroxylate L-Phe to L-Tyr

Class:Id	FailedReaction:5649483
_displayName	Defective PAH does not hydroxylate L-Phe to L-Tyr
_doRelease	TRUE
_timestamp	2017-10-26 20:16:20
authored	[InstanceEdit:5655647] Jassal, Bijay, 2014-12-08
catalystActivity	[CatalystActivity:9631945] phenylalanine 4-monooxygenase activity of PAH S40L:Fe2+ tetramer [cytosol]
compartment	[Compartment:70101] cytosol
created	[InstanceEdit:5649482] Jassal, Bijay, 2014-11-20
disease	[Disease:2160459] phenylketonuria
edited	[InstanceEdit:5655647] Jassal, Bijay, 2014-12-08
entityFunctionalStatus	[EntityFunctionalStatus:5649481] loss_of_function of PAH S40L:Fe2+ tetramer [cytosol]
input	[SimpleEntity:29502] L-Phe [cytosol] [SimpleEntity:29870] BH4 [cytosol] [SimpleEntity:29368] O2 [cytosol]
literatureReference	[LiteratureReference:2160457] Phenylalanine hydroxylase deficiency in a population in Germany: mutational profile and nine novel mutations [LiteratureReference:9027005] Phenylalanine hydroxylase deficiency
modified	[InstanceEdit:5655647] Jassal, Bijay, 2014-12-08 [InstanceEdit:5663028] D'Eustachio, Peter, 2015-01-14 [InstanceEdit:5678275] D'Eustachio, Peter, 2015-02-23 [InstanceEdit:5678599] Jassal, Bijay, 2015-02-25 [InstanceEdit:9027004] D'Eustachio, Peter, 2017-10-26 [InstanceEdit:9631717] Wu, G, 2018-12-12 [InstanceEdit:9830342] Matthews, Lisa, 2023-03-08
name	Defective PAH does not hydroxylate L-Phe to L-Tyr
normalReaction	[Reaction:71118] PAH:Fe2+ tetramer hydroxylates L-Phe to L-Tyr [Homo sapiens]
releaseDate	2015-03-19
reviewed	[InstanceEdit:5668813] D'Eustachio, Peter, 2015-01-28
reviewStatus	[ReviewStatus:9821382] five stars
species	[Species:48887] Homo sapiens
stableIdentifier	[StableIdentifier:5679850] R-HSA-5649483.4
summation	[Summation:179034] Cytosolic phenylalanine hydroxylase (PAH) catalyzes the reac...
(hasEvent)	[Pathway:2160456] Phenylketonuria [Homo sapiens]
(replacementInstances)	[_Deleted:5663029] Deletion of instance: 2160489 [_Deleted:5663030] Deletion of instance: 2160461 [_Deleted:5663032] Deletion of instance: 2160461
(updatedInstance)	[_UpdateTracker:9778005] Update Tracker - [FailedReaction:5649483] Defective PAH does not hydroxylate L-Phe to L-Tyr - v68:[addCatalystActivity] [_UpdateTracker:9780167] Update Tracker - [FailedReaction:5649483] Defective PAH does not hydroxylate L-Phe to L-Tyr - v63:[add_removeLiteratureReference, modifyText] [_UpdateTracker:9939737] Update Tracker - [FailedReaction:5649483] Defective PAH does not hydroxylate L-Phe to L-Tyr - v92:[modifyText]
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