Reactome | Defective GAA does not hydrolyze lysosomal glycogen

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Defective GAA does not hydrolyze lysosomal glycogen Show undefined attributes

authored	[InstanceEdit:5357603] D'Eustachio, Peter, 2014-03-24
catalystActivity	[CatalystActivity:9631935] alpha-glucosidase activity of GAA mutants [lysosomal lumen]
category	transition
compartment	[Compartment:161320] lysosomal lumen
created	[InstanceEdit:9036730] D'Eustachio, Peter, 2018-02-07
crossReference	[DatabaseIdentifier:12020932] Mondo:0009290
dbId	9036729
deleted	[Deleted:9036736] Deletion of instances: 5357606, 5357600
disease	[Disease:5357596] glycogen storage disease II
displayName	Defective GAA does not hydrolyze lysosomal glycogen
edited	[InstanceEdit:5357603] D'Eustachio, Peter, 2014-03-24
entityFunctionalStatus	[EntityFunctionalStatus:5357601] loss_of_function of GAA mutants [lysosomal lumen]
eventOf	[Pathway:R-HSA-5357609] Glycogen storage disease type II (GAA) - Homo sapiens
input	[Complex:R-HSA-9036726] glycogen-GYG1 dimer [lysosomal lumen] 2 × [SimpleEntity:R-ALL-1605715] H2O [lysosomal lumen]
isChimeric	false
isInDisease	true
isInferred	false
literatureReference	[LiteratureReference:5357552] Simultaneous absence of alpha-1,4-glucosidase and alpha-1,6-glucosidase activities (pH 4) in tissues of children with type II glycogen storage disease [LiteratureReference:5357537] Primary structure and processing of lysosomal alpha-glucosidase; homology with the intestinal sucrase-isomaltase complex [LiteratureReference:5357559] Structural and functional changes of lysosomal acid alpha-glucosidase during intracellular transport and maturation
maxDepth	1
modified	[InstanceEdit:9830342] Matthews, Lisa, 2023-03-08
name	Defective GAA does not hydrolyze lysosomal glycogen Defective GAA does not hydrolyze alpha(1,6) linkages in lysosomal glycogen Defective GAA does not hydrolyze alpha(1,4) linkages in lysosomal glycogen
normalReaction	[Reaction:R-HSA-9036727] GAA hydrolyzes lysosomal glycogen
releaseDate	2015-03-19
reviewStatus	[ReviewStatus:9821382] five stars
reviewed	[InstanceEdit:5358504] Jassal, Bijay, 2014-03-28
schemaClass	FailedReaction
species	[Species:48887] Homo sapiens
stId	R-HSA-9036729
summation	[Summation:5357617] Normally, lysosomal alpha-glucosidase (GAA) catalyzes the hy...
updateTrackers	[UpdateTracker:9778507] Update Tracker - [FailedReaction:9036729] Defective GAA does not hydrolyze lysosomal glycogen - v68:[addCatalystActivity]

Referrals

(updatedInstance)	[UpdateTracker:9778507] Update Tracker - [FailedReaction:9036729] Defective GAA does not hydrolyze lysosomal glycogen - v68:[addCatalystActivity]
(replacementInstances)	[Deleted:9036736] Deletion of instances: 5357606, 5357600
(hasEvent)	[Pathway:R-HSA-5357609] Glycogen storage disease type II (GAA)

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