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Schema
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Reaction
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Entries
Hh processing variants are translocated to the cytosol in a VCP-dependent manner
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authored
[InstanceEdit:5359176] Rothfels, Karen, 2014-04-08
catalystActivity
[CatalystActivity:5387377] ABC-type transporter activity of ub-SHH processing variants:ERLEC:OS9:SEL1:SYVN1 dimer:DERL2:VCP hexamer [endoplasmic reticulum membrane]
category
transition
compartment
[Compartment:70101] cytosol
created
[InstanceEdit:5387388] Rothfels, Karen, 2014-04-29
dbId
5387389
disease
[Disease:5358398] holoprosencephaly
displayName
Hh processing variants are translocated to the cytosol in a VCP-dependent manner
edited
[InstanceEdit:5490367] D'Eustachio, Peter, 2014-05-19
entityFunctionalStatus
[EntityFunctionalStatus:9630078] gain_of_function of ub-SHH processing variants:ERLEC:OS9:SEL1:SYVN1 dimer:DERL2:VCP hexamer [endoplasmic reticulum membrane]
eventOf
[Pathway:R-HSA-5362768] Hh mutants are degraded by ERAD - Homo sapiens
followingEvent
[BlackBoxEvent:R-HSA-5387392] processing defective Hh variants are degraded by the proteasome - Homo sapiens
input
6 ×
[SimpleEntity:R-ALL-113592] ATP [cytosol]
6 ×
[SimpleEntity:R-ALL-29356] H2O [cytosol]
[Complex:R-HSA-5483239] ub-SHH processing variants:ERLEC:OS9:SEL1:SYVN1 dimer:DERL2:VCP hexamer [endoplasmic reticulum membrane]
isChimeric
false
isInDisease
true
isInferred
false
literatureReference
[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:5359146] Derlin2 protein facilitates HRD1-mediated retro-translocation of sonic hedgehog at the endoplasmic reticulum
modified
[InstanceEdit:9830342] Matthews, Lisa, 2023-03-08
name
Hh processing variants are translocated to the cytosol in a VCP-dependent manner
output
6 ×
[SimpleEntity:R-ALL-29370] ADP [cytosol]
[DefinedSet:R-HSA-5362377] OS9/ERLEC1 [endoplasmic reticulum quality control compartment]
6 ×
[SimpleEntity:R-ALL-1132287] phosphate [cytoplasm]
[Complex:R-HSA-5362378] SEL1:SYVN1 dimer:DERL2:VCP hexamer [endoplasmic reticulum membrane]
[CandidateSet:R-HSA-5387369] ub-SHH variants [cytosol]
precedingEvent
[Reaction:R-HSA-5483238] Hh processing variants are ubiquitinated - Homo sapiens
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-5387389
summation
[Summation:5387384] Depletion of the ATPase VCP results in the stabilization of ...
Referrals
(hasEvent)
[Pathway:R-HSA-5362768] Hh mutants are degraded by ERAD
(precedingEvent)
[BlackBoxEvent:R-HSA-5387392] processing defective Hh variants are degraded by the proteasome
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