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Schema
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Reaction
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Entries
Hh processing variants bind lectins
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authored
[InstanceEdit:5358461] Rothfels, Karen, 2014-03-28
category
binding
compartment
[Compartment:12045] endoplasmic reticulum membrane
created
[InstanceEdit:5362451] Rothfels, Karen, 2014-04-15
dbId
5362450
disease
[Disease:5358398] holoprosencephaly
displayName
Hh processing variants bind lectins
edited
[InstanceEdit:5490367] D'Eustachio, Peter, 2014-05-19
entityFunctionalStatus
[EntityFunctionalStatus:5362402] gain_of_function of SHH variants [endoplasmic reticulum lumen]
eventOf
[Pathway:R-HSA-5362768] Hh mutants are degraded by ERAD - Homo sapiens
followingEvent
[Reaction:R-HSA-5387386] Hh processing variants are recruited to SEL1:SYVN at the ER membrane - Homo sapiens
input
[DefinedSet:R-HSA-5362377] OS9/ERLEC1 [endoplasmic reticulum quality control compartment]
[CandidateSet:R-HSA-5358456] SHH variants [endoplasmic reticulum lumen]
isChimeric
false
isInDisease
true
isInferred
false
literatureReference
[LiteratureReference:5359146] Derlin2 protein facilitates HRD1-mediated retro-translocation of sonic hedgehog at the endoplasmic reticulum
[LiteratureReference:5358239] Processing and turnover of the Hedgehog protein in the endoplasmic reticulum
[LiteratureReference:5359171] One step at a time: endoplasmic reticulum-associated degradation
[LiteratureReference:5359168] OS-9 and GRP94 deliver mutant alpha1-antitrypsin to the Hrd1-SEL1L ubiquitin ligase complex for ERAD
[LiteratureReference:5359174] SEL1L nucleates a protein complex required for dislocation of misfolded glycoproteins
[LiteratureReference:5359157] Human XTP3-B forms an endoplasmic reticulum quality control scaffold with the HRD1-SEL1L ubiquitin ligase complex and BiP
[LiteratureReference:5359150] Human OS-9, a lectin required for glycoprotein endoplasmic reticulum-associated degradation, recognizes mannose-trimmed N-glycans
modified
[InstanceEdit:9830342] Matthews, Lisa, 2023-03-08
name
Hh processing variants bind lectins
output
[Complex:R-HSA-5362380] SHH processing variants:OS9/ERLEC1 [endoplasmic reticulum lumen]
releaseDate
2014-06-10
reviewStatus
[ReviewStatus:9821382] five stars
reviewed
[InstanceEdit:5483156] Liu, Yulu Cherry, 2014-05-16
schemaClass
Reaction
species
[Species:48887] Homo sapiens
stId
R-HSA-5362450
summation
[Summation:5362795] Like the WT C-terminal fragment of Hh, processing-defective ...
Referrals
(hasEvent)
[Pathway:R-HSA-5362768] Hh mutants are degraded by ERAD
(precedingEvent)
[Reaction:R-HSA-5387386] Hh processing variants are recruited to SEL1:SYVN at the ER membrane
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