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Schema
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Entries
Defective CHST14 causes EDS, musculocontractural type
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authored
[InstanceEdit:3560797] Jassal, B, 2013-05-21
created
[InstanceEdit:3595173] Jassal, B, 2013-05-21
crossReference
[DatabaseIdentifier:12020889] Mondo:0020066
dbId
3595174
disease
[Disease:3656228] Ehlers-Danlos syndrome
displayName
Defective CHST14 causes EDS, musculocontractural type
edited
[InstanceEdit:3560797] Jassal, B, 2013-05-21
eventOf
[Pathway:R-HSA-3560782] Diseases associated with glycosaminoglycan metabolism - Homo sapiens
hasDiagram
true
hasEHLD
false
hasEvent
[FailedReaction:R-HSA-3636919] Defective CHST14 does not transfer SO4(2-) to GalNAc in dermatan or DS - Homo sapiens
isInDisease
true
isInferred
false
lastUpdatedDate
2019-03-25
literatureReference
[LiteratureReference:3636916] Ehlers-Danlos syndromes: revised nosology, Villefranche, 1997. Ehlers-Danlos National Foundation (USA) and Ehlers-Danlos Support Group (UK)
[LiteratureReference:3636912] Musculocontractural Ehlers-Danlos Syndrome (former EDS type VIB) and adducted thumb clubfoot syndrome (ATCS) represent a single clinical entity caused by mutations in the dermatan-4-sulfotransferase 1 encoding CHST14 gene
maxDepth
2
modified
[InstanceEdit:9953280] Stephan, Ralf, 2025-06-02
name
Defective CHST14 causes EDS, musculocontractural type
Defective CHST14 causes Ehlers-Danlos syndrome musculocontractural type
normalPathway
[Pathway:R-HSA-2022923] DS-GAG biosynthesis
releaseDate
2014-09-30
reviewStatus
[ReviewStatus:9821382] five stars
reviewed
[InstanceEdit:5607064] Spillmann, Dorothe, 2014-07-09
schemaClass
Pathway
species
[Species:48887] Homo sapiens
stId
R-HSA-3595174
summation
[Summation:3636870] Carbohydrate sulfotransferase 14 (CHST14 also known as D4ST-...
updateTrackers
[UpdateTracker:9778478] Update Tracker - [Pathway:3595174] Defective CHST14 causes EDS, musculocontractural type - v68:[updateContainedRLE]
[UpdateTracker:9785276] Update Tracker - [Pathway:3595174] Defective CHST14 causes EDS, musculocontractural type - v52:[removeHasEvent]
Referrals
(updatedInstance)
[UpdateTracker:9778478] Update Tracker - [Pathway:3595174] Defective CHST14 causes EDS, musculocontractural type - v68:[updateContainedRLE]
[UpdateTracker:9785276] Update Tracker - [Pathway:3595174] Defective CHST14 causes EDS, musculocontractural type - v52:[removeHasEvent]
(hasEvent)
[Pathway:R-HSA-3560782] Diseases associated with glycosaminoglycan metabolism
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