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Details on Person Defective procollagen type I bind to form an abnormal procollagen triple helix

Class:Id	Reaction:9015582
_displayName	Defective procollagen type I bind to form an abnormal procollagen triple helix
_timestamp	2017-08-17 13:01:51
catalystActivity	[CatalystActivity:2025776] peptidyl-prolyl cis-trans isomerase activity of LEPRE1:PPIB:CRTAP [endoplasmic reticulum lumen]
compartment	[Compartment:17957] endoplasmic reticulum lumen
created	[InstanceEdit:9015580] Varusai, Thawfeek, 2017-08-10
disease	[Disease:9015256] osteogenesis imperfecta
input	[DefinedSet:9015591] Defective COL1A1 procollagen C-linked trimers [endoplasmic reticulum lumen] [Homo sapiens] [EntityWithAccessionedSequence:2127480] SERPINH1 [endoplasmic reticulum lumen] [Homo sapiens]
literatureReference	[LiteratureReference:9015250] The human type I collagen mutation database [LiteratureReference:9015589] Mutations in type I collagen genes resulting in osteogenesis imperfecta in humans
modified	[InstanceEdit:9015624] Varusai, Thawfeek, 2017-08-10 [InstanceEdit:9015629] Varusai, Thawfeek, 2017-08-10 [InstanceEdit:9017214] Varusai, Thawfeek, 2017-08-17
name	Defective procollagen type I bind to form an abnormal procollagen triple helix
normalReaction	[Reaction:2022073] Procollagen triple helix formation [Homo sapiens]
output	[Complex:9015573] Defective COL1A1 collagen and procollagen triple helices:Serpin H1 [endoplasmic reticulum lumen] [Homo sapiens]
precedingEvent	[Reaction:9015565] Defective procollagen type I [Homo sapiens] [BlackBoxEvent:8948231] COLGALT1,COLGALT2:Galactosyl-hydroxylysyl collagen propeptides dissociates [Homo sapiens] [BlackBoxEvent:8948232] Lysyl hydroxylated collagen propeptides dissociate from Lysyl hydroxylases [Homo sapiens] [BlackBoxEvent:8948234] P4HB:4-Hyp collagen propeptides dissociates [Homo sapiens] [BlackBoxEvent:8948222] PLOD3:Fe2+ dimer:Galactosyl-hydroxylysyl collagen propeptides dissociates [Homo sapiens] [BlackBoxEvent:8948224] PLOD3:Fe2+ dimer:Glucosyl-galactosyl-hydroxylysyl collagen propeptides dissociates [Homo sapiens] [BlackBoxEvent:8948226] Prolyl 3-hydroxylases:Fe2+:3,4-Hyp collagen propeptides dissociates [Homo sapiens]
species	[Species:48887] Homo sapiens
stableIdentifier	[StableIdentifier:9015602] R-HSA-9015582.1
summation	[Summation:9015630] Type 1 collagen is a heterotrimer composed of two Collagen a...
(hasEvent)	[Pathway:9013594] Disorders of collagen biosynthesis and modifying enzymes [Homo sapiens]
(precedingEvent)	[BlackBoxEvent:9015574] Secretion of abnormal COL1A1 collagens [Homo sapiens]
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No pathways have been reviewed or authored by Defective procollagen type I bind to form an abnormal procollagen triple helix (9015582)