IRE1 signaling affects cell fate during the unfolded protein response

Endoplasmic reticulum (ER) stress activates a set of signaling pathways, collectively termed the unfolded protein response (UPR). The three UPR branches (IRE1, PERK, and ATF6) promote cell survival by reducing misfolded protein levels. UPR signaling also promotes apoptotic cell death if ER stress is...

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Published in:Science (American Association for the Advancement of Science) Vol. 318; no. 5852; p. 944
Main Authors: Lin, Jonathan H, Li, Han, Yasumura, Douglas, Cohen, Hannah R, Zhang, Chao, Panning, Barbara, Shokat, Kevan M, Lavail, Matthew M, Walter, Peter
Format: Journal Article
Language:English
Published: United States 09.11.2007
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ISSN:1095-9203, 1095-9203
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Abstract Endoplasmic reticulum (ER) stress activates a set of signaling pathways, collectively termed the unfolded protein response (UPR). The three UPR branches (IRE1, PERK, and ATF6) promote cell survival by reducing misfolded protein levels. UPR signaling also promotes apoptotic cell death if ER stress is not alleviated. How the UPR integrates its cytoprotective and proapoptotic outputs to select between life or death cell fates is unknown. We found that IRE1 and ATF6 activities were attenuated by persistent ER stress in human cells. By contrast, PERK signaling, including translational inhibition and proapoptotic transcription regulator Chop induction, was maintained. When IRE1 activity was sustained artificially, cell survival was enhanced, suggesting a causal link between the duration of UPR branch signaling and life or death cell fate after ER stress. Key findings from our studies in cell culture were recapitulated in photoreceptors expressing mutant rhodopsin in animal models of retinitis pigmentosa.
AbstractList Endoplasmic reticulum (ER) stress activates a set of signaling pathways, collectively termed the unfolded protein response (UPR). The three UPR branches (IRE1, PERK, and ATF6) promote cell survival by reducing misfolded protein levels. UPR signaling also promotes apoptotic cell death if ER stress is not alleviated. How the UPR integrates its cytoprotective and proapoptotic outputs to select between life or death cell fates is unknown. We found that IRE1 and ATF6 activities were attenuated by persistent ER stress in human cells. By contrast, PERK signaling, including translational inhibition and proapoptotic transcription regulator Chop induction, was maintained. When IRE1 activity was sustained artificially, cell survival was enhanced, suggesting a causal link between the duration of UPR branch signaling and life or death cell fate after ER stress. Key findings from our studies in cell culture were recapitulated in photoreceptors expressing mutant rhodopsin in animal models of retinitis pigmentosa.
Endoplasmic reticulum (ER) stress activates a set of signaling pathways, collectively termed the unfolded protein response (UPR). The three UPR branches (IRE1, PERK, and ATF6) promote cell survival by reducing misfolded protein levels. UPR signaling also promotes apoptotic cell death if ER stress is not alleviated. How the UPR integrates its cytoprotective and proapoptotic outputs to select between life or death cell fates is unknown. We found that IRE1 and ATF6 activities were attenuated by persistent ER stress in human cells. By contrast, PERK signaling, including translational inhibition and proapoptotic transcription regulator Chop induction, was maintained. When IRE1 activity was sustained artificially, cell survival was enhanced, suggesting a causal link between the duration of UPR branch signaling and life or death cell fate after ER stress. Key findings from our studies in cell culture were recapitulated in photoreceptors expressing mutant rhodopsin in animal models of retinitis pigmentosa.Endoplasmic reticulum (ER) stress activates a set of signaling pathways, collectively termed the unfolded protein response (UPR). The three UPR branches (IRE1, PERK, and ATF6) promote cell survival by reducing misfolded protein levels. UPR signaling also promotes apoptotic cell death if ER stress is not alleviated. How the UPR integrates its cytoprotective and proapoptotic outputs to select between life or death cell fates is unknown. We found that IRE1 and ATF6 activities were attenuated by persistent ER stress in human cells. By contrast, PERK signaling, including translational inhibition and proapoptotic transcription regulator Chop induction, was maintained. When IRE1 activity was sustained artificially, cell survival was enhanced, suggesting a causal link between the duration of UPR branch signaling and life or death cell fate after ER stress. Key findings from our studies in cell culture were recapitulated in photoreceptors expressing mutant rhodopsin in animal models of retinitis pigmentosa.
Author Shokat, Kevan M
Lin, Jonathan H
Panning, Barbara
Zhang, Chao
Li, Han
Cohen, Hannah R
Walter, Peter
Lavail, Matthew M
Yasumura, Douglas
Author_xml – sequence: 1
  givenname: Jonathan H
  surname: Lin
  fullname: Lin, Jonathan H
  email: Jonathan.Lin@ucsf.edu
  organization: Howard Hughes Medical Institute, University of California at San Francisco, San Francisco, CA 94158, USA. Jonathan.Lin@ucsf.edu
– sequence: 2
  givenname: Han
  surname: Li
  fullname: Li, Han
– sequence: 3
  givenname: Douglas
  surname: Yasumura
  fullname: Yasumura, Douglas
– sequence: 4
  givenname: Hannah R
  surname: Cohen
  fullname: Cohen, Hannah R
– sequence: 5
  givenname: Chao
  surname: Zhang
  fullname: Zhang, Chao
– sequence: 6
  givenname: Barbara
  surname: Panning
  fullname: Panning, Barbara
– sequence: 7
  givenname: Kevan M
  surname: Shokat
  fullname: Shokat, Kevan M
– sequence: 8
  givenname: Matthew M
  surname: Lavail
  fullname: Lavail, Matthew M
– sequence: 9
  givenname: Peter
  surname: Walter
  fullname: Walter, Peter
BackLink https://www.ncbi.nlm.nih.gov/pubmed/17991856$$D View this record in MEDLINE/PubMed
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Snippet Endoplasmic reticulum (ER) stress activates a set of signaling pathways, collectively termed the unfolded protein response (UPR). The three UPR branches (IRE1,...
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StartPage 944
SubjectTerms Activating Transcription Factor 6 - metabolism
Animals
Animals, Genetically Modified
Apoptosis
Cell Line
Cell Survival
Disease Models, Animal
eIF-2 Kinase - metabolism
Endoplasmic Reticulum - metabolism
Endoribonucleases - genetics
Endoribonucleases - metabolism
Humans
Kinetics
Membrane Proteins - genetics
Membrane Proteins - metabolism
Mice
Mutation
Protein Folding
Protein Serine-Threonine Kinases - genetics
Protein Serine-Threonine Kinases - metabolism
Proteins - chemistry
Proteins - metabolism
Rats
Retina - metabolism
Retinitis Pigmentosa - metabolism
Rhodopsin - chemistry
Rhodopsin - metabolism
Signal Transduction
Title IRE1 signaling affects cell fate during the unfolded protein response
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