Reactome | Defective SI does not hydrolyze maltotriose

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Defective SI does not hydrolyze maltotriose Show undefined attributes

authored	[InstanceEdit:5675275] D'Eustachio, Peter, 2015-02-15
catalystActivity	[CatalystActivity:9631884] alpha-1,4-glucosidase activity of SI mutant dimers [plasma membrane]
category	transition
compartment	[Compartment:984] extracellular region [Compartment:876] plasma membrane
created	[InstanceEdit:5659876] D'Eustachio, Peter, 2014-12-23
crossReference	[DatabaseIdentifier:12020943] Mondo:0004905
dbId	5659899
disease	[Disease:5659880] intestinal disaccharidase deficiency
displayName	Defective SI does not hydrolyze maltotriose
edited	[InstanceEdit:5675275] D'Eustachio, Peter, 2015-02-15
entityFunctionalStatus	[EntityFunctionalStatus:5659891] loss_of_function of SI mutant dimers [plasma membrane]
eventOf	[Pathway:R-HSA-5659898] Intestinal saccharidase deficiencies - Homo sapiens
inferredFrom	[FailedReaction:R-HSA-5659922] Defective SI does not hydrolyze Mal - Homo sapiens
input	[SimpleEntity:R-ALL-109276] H2O [extracellular region] [SimpleEntity:R-ALL-191103] maltotriose [extracellular region]
isInDisease	true
isInferred	true
literatureReference	[LiteratureReference:5659889] Novel mutations in the human sucrase-isomaltase gene (SI) that cause congenital carbohydrate malabsorption [LiteratureReference:5659866] Structural basis for substrate selectivity in human maltase-glucoamylase and sucrase-isomaltase N-terminal domains
maxDepth	1
modified	[InstanceEdit:9830342] Matthews, Lisa, 2023-03-08
name	Defective SI does not hydrolyze maltotriose
normalReaction	[Reaction:R-HSA-191101] maltotriose + H2O => maltose + D-glucose (sucrase-isomaltase)
releaseDate	2015-03-19
reviewStatus	[ReviewStatus:9821382] five stars
reviewed	[InstanceEdit:5675273] Naim, Hassan Y, Amiri, Mahdi, 2015-02-15 [InstanceEdit:5668911] Jassal, Bijay, 2015-01-29
schemaClass	FailedReaction
species	[Species:48887] Homo sapiens
stId	R-HSA-5659899
summation	[Summation:5659916] Mutations that disrupt the catalytic activity or strongly in...
updateTrackers	[UpdateTracker:9778525] Update Tracker - [FailedReaction:5659899] Defective SI does not hydrolyze maltotriose - v68:[addCatalystActivity]

Referrals

(updatedInstance)	[UpdateTracker:9778525] Update Tracker - [FailedReaction:5659899] Defective SI does not hydrolyze maltotriose - v68:[addCatalystActivity]
(hasEvent)	[Pathway:R-HSA-5659898] Intestinal saccharidase deficiencies
(inferredTo)	[FailedReaction:R-HSA-5659922] Defective SI does not hydrolyze Mal

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