ATF6 is required for efficient rhodopsin clearance and retinal homeostasis in the P23H rho retinitis pigmentosa mouse model

Retinitis Pigmentosa (RP) is a blinding disease that arises from loss of rods and subsequently cones. The P23H rhodopsin knock-in (P23H-KI) mouse develops retinal degeneration that mirrors RP phenotype in patients carrying the orthologous variant. Previously, we found that the P23H rhodopsin protein...

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Published in:Scientific reports Vol. 11; no. 1; pp. 16356 - 14
Main Authors: Lee, Eun-Jin, Chan, Priscilla, Chea, Leon, Kim, Kyle, Kaufman, Randal J., Lin, Jonathan H.
Format: Journal Article
Language:English
Published: London Nature Publishing Group UK 11.08.2021
Nature Publishing Group
Nature Portfolio
Subjects:
631/45
692/4017
692/420
Activating transcription factor 6
Activating Transcription Factor 6 - metabolism
Animals
Cones
Disease Models, Animal
Female
Homeostasis
Homeostasis - physiology
Humanities and Social Sciences
Male
Mice
Mice, Inbred C57BL
multidisciplinary
Phenotypes
Photoreceptors
Protein folding
Proteins
Retina
Retina - metabolism
Retinal degeneration
Retinal Degeneration - metabolism
Retinal Rod Photoreceptor Cells - metabolism
Retinitis
Retinitis pigmentosa
Retinitis Pigmentosa - metabolism
Rhodopsin
Rhodopsin - metabolism
Science
Science (multidisciplinary)
ISSN:2045-2322, 2045-2322
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Abstract Retinitis Pigmentosa (RP) is a blinding disease that arises from loss of rods and subsequently cones. The P23H rhodopsin knock-in (P23H-KI) mouse develops retinal degeneration that mirrors RP phenotype in patients carrying the orthologous variant. Previously, we found that the P23H rhodopsin protein was degraded in P23H-KI retinas, and the Unfolded Protein Response (UPR) promoted P23H rhodopsin degradation in heterologous cells in vitro. Here, we investigated the role of a UPR regulator gene, activating transcription factor 6 ( Atf6 ), in rhodopsin protein homeostasis in heterozygous P23H rhodopsin ( Rho + /P23H ) mice. Significantly increased rhodopsin protein levels were found in Atf6 −/− Rho + /P23H retinas compared to Atf6 + /− Rho + /P23H retinas at early ages (~ P12), while rhodopsin mRNA levels were not different. The IRE1 pathway of the UPR was hyper-activated in young Atf6 −/− Rho + /P23H retinas, and photoreceptor layer thickness was unchanged at this early age in Rho + /P23H mice lacking Atf6 . By contrast, older Atf6 −/− Rho + /P23H mice developed significantly increased retinal degeneration in comparison to Atf6 + /− Rho + /P23H mice in all retinal layers, accompanied by reduced rhodopsin protein levels. Our findings demonstrate that Atf6 is required for efficient clearance of rhodopsin protein in rod photoreceptors expressing P23H rhodopsin, and that loss of Atf6 ultimately accelerates retinal degeneration in P23H-KI mice.
AbstractList Retinitis Pigmentosa (RP) is a blinding disease that arises from loss of rods and subsequently cones. The P23H rhodopsin knock-in (P23H-KI) mouse develops retinal degeneration that mirrors RP phenotype in patients carrying the orthologous variant. Previously, we found that the P23H rhodopsin protein was degraded in P23H-KI retinas, and the Unfolded Protein Response (UPR) promoted P23H rhodopsin degradation in heterologous cells in vitro. Here, we investigated the role of a UPR regulator gene, activating transcription factor 6 (Atf6), in rhodopsin protein homeostasis in heterozygous P23H rhodopsin (Rho+/P23H) mice. Significantly increased rhodopsin protein levels were found in Atf6−/−Rho+/P23H retinas compared to Atf6+/−Rho+/P23H retinas at early ages (~ P12), while rhodopsin mRNA levels were not different. The IRE1 pathway of the UPR was hyper-activated in young Atf6−/−Rho+/P23H retinas, and photoreceptor layer thickness was unchanged at this early age in Rho+/P23H mice lacking Atf6. By contrast, older Atf6−/−Rho+/P23H mice developed significantly increased retinal degeneration in comparison to Atf6+/−Rho+/P23H mice in all retinal layers, accompanied by reduced rhodopsin protein levels. Our findings demonstrate that Atf6 is required for efficient clearance of rhodopsin protein in rod photoreceptors expressing P23H rhodopsin, and that loss of Atf6 ultimately accelerates retinal degeneration in P23H-KI mice.
Retinitis Pigmentosa (RP) is a blinding disease that arises from loss of rods and subsequently cones. The P23H rhodopsin knock-in (P23H-KI) mouse develops retinal degeneration that mirrors RP phenotype in patients carrying the orthologous variant. Previously, we found that the P23H rhodopsin protein was degraded in P23H-KI retinas, and the Unfolded Protein Response (UPR) promoted P23H rhodopsin degradation in heterologous cells in vitro. Here, we investigated the role of a UPR regulator gene, activating transcription factor 6 (Atf6), in rhodopsin protein homeostasis in heterozygous P23H rhodopsin (Rho+/P23H) mice. Significantly increased rhodopsin protein levels were found in Atf6-/-Rho+/P23H retinas compared to Atf6+/-Rho+/P23H retinas at early ages (~ P12), while rhodopsin mRNA levels were not different. The IRE1 pathway of the UPR was hyper-activated in young Atf6-/-Rho+/P23H retinas, and photoreceptor layer thickness was unchanged at this early age in Rho+/P23H mice lacking Atf6. By contrast, older Atf6-/-Rho+/P23H mice developed significantly increased retinal degeneration in comparison to Atf6+/-Rho+/P23H mice in all retinal layers, accompanied by reduced rhodopsin protein levels. Our findings demonstrate that Atf6 is required for efficient clearance of rhodopsin protein in rod photoreceptors expressing P23H rhodopsin, and that loss of Atf6 ultimately accelerates retinal degeneration in P23H-KI mice.Retinitis Pigmentosa (RP) is a blinding disease that arises from loss of rods and subsequently cones. The P23H rhodopsin knock-in (P23H-KI) mouse develops retinal degeneration that mirrors RP phenotype in patients carrying the orthologous variant. Previously, we found that the P23H rhodopsin protein was degraded in P23H-KI retinas, and the Unfolded Protein Response (UPR) promoted P23H rhodopsin degradation in heterologous cells in vitro. Here, we investigated the role of a UPR regulator gene, activating transcription factor 6 (Atf6), in rhodopsin protein homeostasis in heterozygous P23H rhodopsin (Rho+/P23H) mice. Significantly increased rhodopsin protein levels were found in Atf6-/-Rho+/P23H retinas compared to Atf6+/-Rho+/P23H retinas at early ages (~ P12), while rhodopsin mRNA levels were not different. The IRE1 pathway of the UPR was hyper-activated in young Atf6-/-Rho+/P23H retinas, and photoreceptor layer thickness was unchanged at this early age in Rho+/P23H mice lacking Atf6. By contrast, older Atf6-/-Rho+/P23H mice developed significantly increased retinal degeneration in comparison to Atf6+/-Rho+/P23H mice in all retinal layers, accompanied by reduced rhodopsin protein levels. Our findings demonstrate that Atf6 is required for efficient clearance of rhodopsin protein in rod photoreceptors expressing P23H rhodopsin, and that loss of Atf6 ultimately accelerates retinal degeneration in P23H-KI mice.
Abstract Retinitis Pigmentosa (RP) is a blinding disease that arises from loss of rods and subsequently cones. The P23H rhodopsin knock-in (P23H-KI) mouse develops retinal degeneration that mirrors RP phenotype in patients carrying the orthologous variant. Previously, we found that the P23H rhodopsin protein was degraded in P23H-KI retinas, and the Unfolded Protein Response (UPR) promoted P23H rhodopsin degradation in heterologous cells in vitro. Here, we investigated the role of a UPR regulator gene, activating transcription factor 6 (Atf6), in rhodopsin protein homeostasis in heterozygous P23H rhodopsin (Rho +/P23H ) mice. Significantly increased rhodopsin protein levels were found in Atf6 −/− Rho +/P23H retinas compared to Atf6 +/− Rho +/P23H retinas at early ages (~ P12), while rhodopsin mRNA levels were not different. The IRE1 pathway of the UPR was hyper-activated in young Atf6 −/− Rho +/P23H retinas, and photoreceptor layer thickness was unchanged at this early age in Rho +/P23H mice lacking Atf6. By contrast, older Atf6 −/− Rho +/P23H mice developed significantly increased retinal degeneration in comparison to Atf6 +/− Rho +/P23H mice in all retinal layers, accompanied by reduced rhodopsin protein levels. Our findings demonstrate that Atf6 is required for efficient clearance of rhodopsin protein in rod photoreceptors expressing P23H rhodopsin, and that loss of Atf6 ultimately accelerates retinal degeneration in P23H-KI mice.
Retinitis Pigmentosa (RP) is a blinding disease that arises from loss of rods and subsequently cones. The P23H rhodopsin knock-in (P23H-KI) mouse develops retinal degeneration that mirrors RP phenotype in patients carrying the orthologous variant. Previously, we found that the P23H rhodopsin protein was degraded in P23H-KI retinas, and the Unfolded Protein Response (UPR) promoted P23H rhodopsin degradation in heterologous cells in vitro. Here, we investigated the role of a UPR regulator gene, activating transcription factor 6 ( Atf6 ), in rhodopsin protein homeostasis in heterozygous P23H rhodopsin ( Rho + /P23H ) mice. Significantly increased rhodopsin protein levels were found in Atf6 −/− Rho + /P23H retinas compared to Atf6 + /− Rho + /P23H retinas at early ages (~ P12), while rhodopsin mRNA levels were not different. The IRE1 pathway of the UPR was hyper-activated in young Atf6 −/− Rho + /P23H retinas, and photoreceptor layer thickness was unchanged at this early age in Rho + /P23H mice lacking Atf6 . By contrast, older Atf6 −/− Rho + /P23H mice developed significantly increased retinal degeneration in comparison to Atf6 + /− Rho + /P23H mice in all retinal layers, accompanied by reduced rhodopsin protein levels. Our findings demonstrate that Atf6 is required for efficient clearance of rhodopsin protein in rod photoreceptors expressing P23H rhodopsin, and that loss of Atf6 ultimately accelerates retinal degeneration in P23H-KI mice.
Retinitis Pigmentosa (RP) is a blinding disease that arises from loss of rods and subsequently cones. The P23H rhodopsin knock-in (P23H-KI) mouse develops retinal degeneration that mirrors RP phenotype in patients carrying the orthologous variant. Previously, we found that the P23H rhodopsin protein was degraded in P23H-KI retinas, and the Unfolded Protein Response (UPR) promoted P23H rhodopsin degradation in heterologous cells in vitro. Here, we investigated the role of a UPR regulator gene, activating transcription factor 6 (Atf6), in rhodopsin protein homeostasis in heterozygous P23H rhodopsin (Rho ) mice. Significantly increased rhodopsin protein levels were found in Atf6 Rho retinas compared to Atf6 Rho retinas at early ages (~ P12), while rhodopsin mRNA levels were not different. The IRE1 pathway of the UPR was hyper-activated in young Atf6 Rho retinas, and photoreceptor layer thickness was unchanged at this early age in Rho mice lacking Atf6. By contrast, older Atf6 Rho mice developed significantly increased retinal degeneration in comparison to Atf6 Rho mice in all retinal layers, accompanied by reduced rhodopsin protein levels. Our findings demonstrate that Atf6 is required for efficient clearance of rhodopsin protein in rod photoreceptors expressing P23H rhodopsin, and that loss of Atf6 ultimately accelerates retinal degeneration in P23H-KI mice.
ArticleNumber 16356
Author Chea, Leon
Kim, Kyle
Lee, Eun-Jin
Kaufman, Randal J.
Lin, Jonathan H.
Chan, Priscilla
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– sequence: 2
  givenname: Priscilla
  surname: Chan
  fullname: Chan, Priscilla
  organization: Department of Neurology, Keck School of Medicine, University of Southern California
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  givenname: Leon
  surname: Chea
  fullname: Chea, Leon
  organization: Department of Ophthalmology, Stanford University, Department of Pathology, Stanford University, VA Palo Alto Healthcare System
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  givenname: Kyle
  surname: Kim
  fullname: Kim, Kyle
  organization: Department of Ophthalmology, Stanford University, Department of Pathology, Stanford University, VA Palo Alto Healthcare System
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  email: jlinn@stanford.edu
  organization: Department of Ophthalmology, Stanford University, Department of Pathology, Stanford University, VA Palo Alto Healthcare System, School of Medicine, Stanford University
BackLink https://www.ncbi.nlm.nih.gov/pubmed/34381136$$D View this record in MEDLINE/PubMed
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Cites_doi 10.1038/nrm2199
10.1016/s1097-2765(00)00133-7
10.1073/pnas.91.3.974
10.1091/mbc.E11-08-0663
10.1007/s12035-015-9456-z
10.1002/dvdy.389
10.1007/978-3-319-17121-0_25
10.1016/0092-8674(93)90648-a
10.1172/jci.insight.136041
10.1038/415092a
10.1101/gad.14.2.152
10.1038/ncb0311-184
10.1016/0896-6273(92)90236-7
10.1038/ng.3319
10.1038/sj.eye.6701573
10.1126/science.1209038
10.1002/cne.22800
10.1076/opge.22.3.133.2224
10.1007/s12035-014-8881-8
10.7554/eLife.46595
10.1002/jcp.21152
10.1074/jbc.REV120.010218
10.1016/j.exer.2017.10.023
10.1016/j.preteyeres.2018.03.005
10.1074/jbc.M406685200
10.1007/s00125-012-2809-5
10.1371/journal.pgen.1009172
10.1371/journal.pone.0083871
10.1167/iovs.15-16778
10.1159/000446321
10.1021/bi00186a011
10.1016/S0140-6736(06)69740-7
10.1046/j.1471-4159.2001.00623.x
10.1073/pnas.1606387114
10.1074/jbc.M204955200
10.1007/s00439-015-1571-4
10.1056/NEJM199011083231903
10.1128/mcb.23.21.7448-7459.2003
10.7554/eLife.11878
10.1091/mbc.10.11.3787
10.1161/01.RES.0000220643.65941.8d
10.1016/j.ajpath.2012.12.020
10.7554/eLife.15550
10.1038/16729
10.1093/hmg/ddt561
10.1007/978-1-4614-0631-0_71
10.1080/15548627.2018.1463121
10.1038/s41467-018-08129-2
10.1167/iovs.12-10222
10.1016/j.devcel.2007.07.005
10.1016/j.ceb.2004.09.012
10.7554/eLife.37168
10.1167/iovs.15-16969
10.1016/j.celrep.2013.03.024
10.1038/ejhg.2017.131
10.7554/eLife.11880
10.1016/j.preteyeres.2010.03.004
10.1074/jbc.M110.209759
10.1242/jcs.115.14.2907
10.1172/JCI21848
10.1016/s0092-8674(01)00611-0
10.15252/embj.201488208
10.1073/pnas.0911991107
10.1101/cshperspect.a013177
10.1126/scisignal.aan5785
10.1038/s41419-019-1780-1
10.1073/pnas.88.15.6481
10.1247/csf.07044
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References Kohl (CR30) 2015; 47
Qiu, Yao, Jia, Thompson, Zacks (CR56) 2019; 10
Dettoraki, Moschos (CR61) 2016; 56
Blackwood (CR63) 2019; 10
Campian, Qian, Gao, Eaton (CR57) 2004; 279
Skorczyk-Werner (CR46) 2017; 25
Dryja (CR5) 1990; 323
Ron, Harding (CR15) 2012; 4
Berger, Kloeckener-Gruissem, Neidhardt (CR3) 2010; 29
Massoudi (CR51) 2021; 62
Jones, Hagglund, Carlsson (CR73) 2021
Harding, Zhang, Ron (CR14) 1999; 397
Adekeye, Haeri, Solessio, Knox (CR54) 2014; 9
Lee (CR45) 2020
Yao (CR55) 2018; 14
Ansar (CR43) 2015; 134
Verbakel (CR4) 2018; 66
Sakami (CR23) 2011; 286
Chiang (CR31) 2016; 854
Plate (CR67) 2016
Wu (CR41) 2007; 13
Saliba, Munro, Luthert, Cheetham (CR25) 2002; 115
Alavi (CR26) 2015; 56
Chiang (CR22) 2015; 52
Paschen, Frandsen (CR35) 2001; 79
Rao, Bredesen (CR36) 2004; 16
Liu (CR49) 2013; 182
Chiang, Messah, Lin (CR39) 2012; 23
Reimold (CR28) 2000; 14
Lee, Iwakoshi, Glimcher (CR12) 2003; 23
LaVail (CR32) 2018; 167
Azarmina (CR60) 2013; 8
Ji, Zhu, Grzywacz, Lee (CR71) 2012; 520
Haze, Yoshida, Yanagi, Yura, Mori (CR20) 1999; 10
Chiang, Hiramatsu, Messah, Kroeger, Lin (CR27) 2012; 53
Wang (CR7) 2001; 22
Yoshida, Matsui, Yamamoto, Okada, Mori (CR11) 2001; 107
Hartong, Berson, Dryja (CR2) 2006; 368
Tan (CR34) 2001; 42
Olsson (CR33) 1992; 9
Pak, Lee, Craft (CR74) 2015; 21
Kaushal, Khorana (CR38) 1994; 33
Calfon (CR9) 2002; 415
Xu (CR47) 2015; 56
CR50
Berson (CR1) 1993; 34
Kroeger (CR48) 2018; 11
Portera-Cailliau, Sung, Nathans, Adler (CR58) 1994; 91
Zhang (CR29) 2005; 115
Walter, Ron (CR8) 2011; 334
Grandjean, Wiseman (CR69) 2020; 295
Shoulders (CR13) 2013; 3
Komeima, Rogers, Campochiaro (CR59) 2007; 213
Gallagher, Walter (CR65) 2016
Ron, Walter (CR16) 2007; 8
Tabas, Ron (CR18) 2011; 13
Ye (CR21) 2000; 6
Kroeger, Chiang, Lin (CR53) 2012; 723
Torres (CR68) 2019
Adachi (CR40) 2008; 33
Chiang (CR44) 2017; 114
Gorbatyuk (CR42) 2010; 107
Xu, Zhao, Wang (CR52) 2020; 16
Chen (CR19) 2014; 33
Martindale (CR66) 2006; 98
Han (CR17) 2013; 56
Paxman (CR70) 2018
Sakami, Kolesnikov, Kefalov, Palczewski (CR24) 2014; 23
Gallagher (CR64) 2016
Sung (CR6) 1991; 88
Illing, Rajan, Bence, Kopito (CR37) 2002; 277
Roos (CR72) 2016; 53
Holder (CR62) 2004; 18
Cox, Shamu, Walter (CR10) 1993; 73
JJ Martindale (95895_CR66) 2006; 98
J Han (95895_CR17) 2013; 56
MD Shoulders (95895_CR13) 2013; 3
J Wu (95895_CR41) 2007; 13
JE Olsson (95895_CR33) 1992; 9
M Azarmina (95895_CR60) 2013; 8
D Ron (95895_CR16) 2007; 8
A Roos (95895_CR72) 2016; 53
RV Rao (95895_CR36) 2004; 16
CM Gallagher (95895_CR65) 2016
L Plate (95895_CR67) 2016
A Adekeye (95895_CR54) 2014; 9
C Portera-Cailliau (95895_CR58) 1994; 91
WC Chiang (95895_CR22) 2015; 52
WC Chiang (95895_CR39) 2012; 23
95895_CR50
S Sakami (95895_CR23) 2011; 286
WC Chiang (95895_CR27) 2012; 53
J Ye (95895_CR21) 2000; 6
M Xu (95895_CR47) 2015; 56
MS Gorbatyuk (95895_CR42) 2010; 107
K Haze (95895_CR20) 1999; 10
MM LaVail (95895_CR32) 2018; 167
SK Verbakel (95895_CR4) 2018; 66
JS Cox (95895_CR10) 1993; 73
EJ Lee (95895_CR45) 2020
WC Chiang (95895_CR44) 2017; 114
SE Torres (95895_CR68) 2019
JL Campian (95895_CR57) 2004; 279
H Kroeger (95895_CR48) 2018; 11
J Xu (95895_CR52) 2020; 16
AH Lee (95895_CR12) 2003; 23
Y Ji (95895_CR71) 2012; 520
DT Hartong (95895_CR2) 2006; 368
Y Qiu (95895_CR56) 2019; 10
M Calfon (95895_CR9) 2002; 415
EL Berson (95895_CR1) 1993; 34
D Massoudi (95895_CR51) 2021; 62
JMD Grandjean (95895_CR69) 2020; 295
W Berger (95895_CR3) 2010; 29
TP Dryja (95895_CR5) 1990; 323
S Kohl (95895_CR30) 2015; 47
W Paschen (95895_CR35) 2001; 79
I Jones (95895_CR73) 2021
CY Chen (95895_CR19) 2014; 33
K Komeima (95895_CR59) 2007; 213
JS Pak (95895_CR74) 2015; 21
L Liu (95895_CR49) 2013; 182
M Dettoraki (95895_CR61) 2016; 56
I Tabas (95895_CR18) 2011; 13
CH Sung (95895_CR6) 1991; 88
K Zhang (95895_CR29) 2005; 115
GE Holder (95895_CR62) 2004; 18
M Ansar (95895_CR43) 2015; 134
HP Harding (95895_CR14) 1999; 397
EA Blackwood (95895_CR63) 2019; 10
H Kroeger (95895_CR53) 2012; 723
RS Saliba (95895_CR25) 2002; 115
E Tan (95895_CR34) 2001; 42
P Walter (95895_CR8) 2011; 334
MV Alavi (95895_CR26) 2015; 56
A Skorczyk-Werner (95895_CR46) 2017; 25
S Kaushal (95895_CR38) 1994; 33
AM Reimold (95895_CR28) 2000; 14
S Sakami (95895_CR24) 2014; 23
D Ron (95895_CR15) 2012; 4
WC Chiang (95895_CR31) 2016; 854
Q Wang (95895_CR7) 2001; 22
ME Illing (95895_CR37) 2002; 277
CM Gallagher (95895_CR64) 2016
H Yoshida (95895_CR11) 2001; 107
Y Adachi (95895_CR40) 2008; 33
J Yao (95895_CR55) 2018; 14
R Paxman (95895_CR70) 2018
References_xml – volume: 34
  start-page: 1659
  year: 1993
  end-page: 1676
  ident: CR1
  article-title: Retinitis pigmentosa. The Friedenwald Lecture
  publication-title: Invest. Ophthalmol. Vis. Sci.
– volume: 8
  start-page: 519
  year: 2007
  end-page: 529
  ident: CR16
  article-title: Signal integration in the endoplasmic reticulum unfolded protein response
  publication-title: Nat. Rev. Mol. Cell. Biol.
  doi: 10.1038/nrm2199
– volume: 6
  start-page: 1355
  year: 2000
  end-page: 1364
  ident: CR21
  article-title: ER stress induces cleavage of membrane-bound ATF6 by the same proteases that process SREBPs
  publication-title: Mol. Cell
  doi: 10.1016/s1097-2765(00)00133-7
– volume: 91
  start-page: 974
  year: 1994
  end-page: 978
  ident: CR58
  article-title: Apoptotic photoreceptor cell death in mouse models of retinitis pigmentosa
  publication-title: Proc. Natl. Acad. Sci. USA
  doi: 10.1073/pnas.91.3.974
– volume: 8
  start-page: 191
  year: 2013
  end-page: 192
  ident: CR60
  article-title: Full-field versus multifocal electroretinography
  publication-title: J. Ophthalmic Vis. Res.
– volume: 23
  start-page: 758
  year: 2012
  end-page: 770
  ident: CR39
  article-title: IRE1 directs proteasomal and lysosomal degradation of misfolded rhodopsin
  publication-title: Mol. Biol. Cell
  doi: 10.1091/mbc.E11-08-0663
– volume: 53
  start-page: 5527
  year: 2016
  end-page: 5541
  ident: CR72
  article-title: Cellular signature of SIL1 depletion: Disease pathogenesis due to alterations in protein composition beyond the ER machinery
  publication-title: Mol. Neurobiol.
  doi: 10.1007/s12035-015-9456-z
– year: 2021
  ident: CR73
  article-title: Reduced mTORC1-signaling in retinal ganglion cells leads to vascular retinopathy
  publication-title: Dev. Dyn.
  doi: 10.1002/dvdy.389
– volume: 854
  start-page: 185
  year: 2016
  end-page: 191
  ident: CR31
  article-title: Ablation of chop transiently enhances photoreceptor survival but does not prevent retinal degeneration in transgenic mice expressing human P23H rhodopsin
  publication-title: Adv. Exp. Med. Biol.
  doi: 10.1007/978-3-319-17121-0_25
– volume: 73
  start-page: 1197
  year: 1993
  end-page: 1206
  ident: CR10
  article-title: Transcriptional induction of genes encoding endoplasmic reticulum resident proteins requires a transmembrane protein kinase
  publication-title: Cell
  doi: 10.1016/0092-8674(93)90648-a
– year: 2020
  ident: CR45
  article-title: Multiexon deletion alleles of ATF6 linked to achromatopsia
  publication-title: JCI Insight
  doi: 10.1172/jci.insight.136041
– volume: 415
  start-page: 92
  year: 2002
  end-page: 96
  ident: CR9
  article-title: IRE1 couples endoplasmic reticulum load to secretory capacity by processing the XBP-1 mRNA
  publication-title: Nature
  doi: 10.1038/415092a
– volume: 14
  start-page: 152
  year: 2000
  end-page: 157
  ident: CR28
  article-title: An essential role in liver development for transcription factor XBP-1
  publication-title: Genes Dev.
  doi: 10.1101/gad.14.2.152
– volume: 13
  start-page: 184
  year: 2011
  end-page: 190
  ident: CR18
  article-title: Integrating the mechanisms of apoptosis induced by endoplasmic reticulum stress
  publication-title: Nat. Cell. Biol.
  doi: 10.1038/ncb0311-184
– volume: 9
  start-page: 815
  year: 1992
  end-page: 830
  ident: CR33
  article-title: Transgenic mice with a rhodopsin mutation (Pro23His): A mouse model of autosomal dominant retinitis pigmentosa
  publication-title: Neuron
  doi: 10.1016/0896-6273(92)90236-7
– volume: 62
  start-page: 3073
  year: 2021
  ident: CR51
  article-title: The UPR transducer IRE1α is required for photoreceptor health and protection against retinal degeneration
  publication-title: IOVS
– volume: 47
  start-page: 757
  year: 2015
  end-page: 765
  ident: CR30
  article-title: Mutations in the unfolded protein response regulator ATF6 cause the cone dysfunction disorder achromatopsia
  publication-title: Nat. Genet.
  doi: 10.1038/ng.3319
– volume: 18
  start-page: 1133
  year: 2004
  end-page: 1143
  ident: CR62
  article-title: Electrophysiological assessment of optic nerve disease
  publication-title: Eye (Lond)
  doi: 10.1038/sj.eye.6701573
– volume: 334
  start-page: 1081
  year: 2011
  end-page: 1086
  ident: CR8
  article-title: The unfolded protein response: from stress pathway to homeostatic regulation
  publication-title: Science
  doi: 10.1126/science.1209038
– volume: 520
  start-page: 874
  year: 2012
  end-page: 888
  ident: CR71
  article-title: Rearrangement of the cone mosaic in the retina of the rat model of retinitis pigmentosa
  publication-title: J. Comp. Neurol.
  doi: 10.1002/cne.22800
– volume: 22
  start-page: 133
  year: 2001
  end-page: 154
  ident: CR7
  article-title: Update on the molecular genetics of retinitis pigmentosa
  publication-title: Ophthalmic Genet.
  doi: 10.1076/opge.22.3.133.2224
– volume: 52
  start-page: 679
  year: 2015
  end-page: 695
  ident: CR22
  article-title: Robust endoplasmic reticulum-associated degradation of rhodopsin precedes retinal degeneration
  publication-title: Mol. Neurobiol.
  doi: 10.1007/s12035-014-8881-8
– year: 2019
  ident: CR68
  article-title: Ceapins block the unfolded protein response sensor ATF6alpha by inducing a neomorphic inter-organelle tether
  publication-title: Elife
  doi: 10.7554/eLife.46595
– volume: 213
  start-page: 809
  year: 2007
  end-page: 815
  ident: CR59
  article-title: Antioxidants slow photoreceptor cell death in mouse models of retinitis pigmentosa
  publication-title: J. Cell Physiol.
  doi: 10.1002/jcp.21152
– ident: CR50
– volume: 295
  start-page: 15692
  year: 2020
  end-page: 15711
  ident: CR69
  article-title: Small molecule strategies to harness the unfolded protein response: where do we go from here?
  publication-title: J. Biol. Chem.
  doi: 10.1074/jbc.REV120.010218
– volume: 167
  start-page: 56
  year: 2018
  end-page: 90
  ident: CR32
  article-title: Phenotypic characterization of P23H and S334ter rhodopsin transgenic rat models of inherited retinal degeneration
  publication-title: Exp. Eye Res.
  doi: 10.1016/j.exer.2017.10.023
– volume: 66
  start-page: 157
  year: 2018
  end-page: 186
  ident: CR4
  article-title: Non-syndromic retinitis pigmentosa
  publication-title: Prog. Retina Eye Res.
  doi: 10.1016/j.preteyeres.2018.03.005
– volume: 279
  start-page: 46580
  year: 2004
  end-page: 46587
  ident: CR57
  article-title: Oxygen tolerance and coupling of mitochondrial electron transport
  publication-title: J. Biol. Chem.
  doi: 10.1074/jbc.M406685200
– volume: 56
  start-page: 911
  year: 2013
  end-page: 924
  ident: CR17
  article-title: ER stress signalling through eIF2alpha and CHOP, but not IRE1alpha, attenuates adipogenesis in mice
  publication-title: Diabetologia
  doi: 10.1007/s00125-012-2809-5
– volume: 16
  year: 2020
  ident: CR52
  article-title: Suppression of retinal degeneration by two novel ERAD ubiquitin E3 ligases SORDD1/2 in Drosophila
  publication-title: PLoS Genet.
  doi: 10.1371/journal.pgen.1009172
– volume: 9
  year: 2014
  ident: CR54
  article-title: Ablation of the proapoptotic genes CHOP or Ask1 does not prevent or delay loss of visual function in a P23H transgenic mouse model of retinitis pigmentosa
  publication-title: PLoS ONE
  doi: 10.1371/journal.pone.0083871
– volume: 21
  start-page: 1281
  year: 2015
  end-page: 1294
  ident: CR74
  article-title: The retinal phenotype of Grk1-/- is compromised by a Crb1 rd8 mutation
  publication-title: Mol. Vis.
– volume: 56
  start-page: 3889
  year: 2015
  end-page: 3895
  ident: CR47
  article-title: ATF6 is mutated in early onset photoreceptor degeneration with macular involvement
  publication-title: Invest. Ophthalmol. Vis. Sci.
  doi: 10.1167/iovs.15-16778
– volume: 56
  start-page: 169
  year: 2016
  end-page: 177
  ident: CR61
  article-title: The role of multifocal electroretinography in the assessment of drug-induced retinopathy: A review of the literature
  publication-title: Ophthalmic Res.
  doi: 10.1159/000446321
– volume: 33
  start-page: 6121
  year: 1994
  end-page: 6128
  ident: CR38
  article-title: Structure and function in rhodopsin. 7. Point mutations associated with autosomal dominant retinitis pigmentosa
  publication-title: Biochemistry
  doi: 10.1021/bi00186a011
– volume: 368
  start-page: 1795
  year: 2006
  end-page: 1809
  ident: CR2
  article-title: Retinitis pigmentosa
  publication-title: Lancet
  doi: 10.1016/S0140-6736(06)69740-7
– volume: 79
  start-page: 719
  year: 2001
  end-page: 725
  ident: CR35
  article-title: Endoplasmic reticulum dysfunction: A common denominator for cell injury in acute and degenerative diseases of the brain?
  publication-title: J. Neurochem.
  doi: 10.1046/j.1471-4159.2001.00623.x
– volume: 114
  start-page: 400
  year: 2017
  end-page: 405
  ident: CR44
  article-title: Achromatopsia mutations target sequential steps of ATF6 activation
  publication-title: Proc. Natl. Acad. Sci. USA
  doi: 10.1073/pnas.1606387114
– volume: 277
  start-page: 34150
  year: 2002
  end-page: 34160
  ident: CR37
  article-title: A rhodopsin mutant linked to autosomal dominant retinitis pigmentosa is prone to aggregate and interacts with the ubiquitin proteasome system
  publication-title: J. Biol. Chem.
  doi: 10.1074/jbc.M204955200
– volume: 134
  start-page: 941
  year: 2015
  end-page: 950
  ident: CR43
  article-title: Mutation of ATF6 causes autosomal recessive achromatopsia
  publication-title: Hum. Genet.
  doi: 10.1007/s00439-015-1571-4
– volume: 323
  start-page: 1302
  year: 1990
  end-page: 1307
  ident: CR5
  article-title: Mutations within the rhodopsin gene in patients with autosomal dominant retinitis pigmentosa
  publication-title: N. Engl. J. Med.
  doi: 10.1056/NEJM199011083231903
– volume: 23
  start-page: 7448
  year: 2003
  end-page: 7459
  ident: CR12
  article-title: XBP-1 regulates a subset of endoplasmic reticulum resident chaperone genes in the unfolded protein response
  publication-title: Mol. Cell. Biol.
  doi: 10.1128/mcb.23.21.7448-7459.2003
– year: 2016
  ident: CR64
  article-title: Ceapins are a new class of unfolded protein response inhibitors, selectively targeting the ATF6alpha branch
  publication-title: Elife
  doi: 10.7554/eLife.11878
– volume: 10
  start-page: 3787
  year: 1999
  end-page: 3799
  ident: CR20
  article-title: Mammalian transcription factor ATF6 is synthesized as a transmembrane protein and activated by proteolysis in response to endoplasmic reticulum stress
  publication-title: Mol. Biol. Cell
  doi: 10.1091/mbc.10.11.3787
– volume: 98
  start-page: 1186
  year: 2006
  end-page: 1193
  ident: CR66
  article-title: Endoplasmic reticulum stress gene induction and protection from ischemia/reperfusion injury in the hearts of transgenic mice with a tamoxifen-regulated form of ATF6
  publication-title: Circ. Res.
  doi: 10.1161/01.RES.0000220643.65941.8d
– volume: 182
  start-page: 1412
  year: 2013
  end-page: 1424
  ident: CR49
  article-title: Targeting the IRE1alpha/XBP1 and ATF6 arms of the unfolded protein response enhances VEGF blockade to prevent retinal and choroidal neovascularization
  publication-title: Am. J. Pathol.
  doi: 10.1016/j.ajpath.2012.12.020
– year: 2016
  ident: CR67
  article-title: Small molecule proteostasis regulators that reprogram the ER to reduce extracellular protein aggregation
  publication-title: Elife
  doi: 10.7554/eLife.15550
– volume: 397
  start-page: 271
  year: 1999
  end-page: 274
  ident: CR14
  article-title: Protein translation and folding are coupled by an endoplasmic-reticulum-resident kinase
  publication-title: Nature
  doi: 10.1038/16729
– volume: 23
  start-page: 1723
  year: 2014
  end-page: 1741
  ident: CR24
  article-title: P23H opsin knock-in mice reveal a novel step in retinal rod disc morphogenesis
  publication-title: Hum. Mol. Genet.
  doi: 10.1093/hmg/ddt561
– volume: 723
  start-page: 559
  year: 2012
  end-page: 565
  ident: CR53
  article-title: Endoplasmic reticulum-associated degradation (ERAD) of misfolded glycoproteins and mutant P23H rhodopsin in photoreceptor cells
  publication-title: Adv. Exp. Med. Biol.
  doi: 10.1007/978-1-4614-0631-0_71
– volume: 14
  start-page: 1226
  year: 2018
  end-page: 1238
  ident: CR55
  article-title: Inhibiting autophagy reduces retinal degeneration caused by protein misfolding
  publication-title: Autophagy
  doi: 10.1080/15548627.2018.1463121
– volume: 10
  start-page: 187
  year: 2019
  ident: CR63
  article-title: Pharmacologic ATF6 activation confers global protection in widespread disease models by reprograming cellular proteostasis
  publication-title: Nat. Commun.
  doi: 10.1038/s41467-018-08129-2
– volume: 53
  start-page: 7159
  year: 2012
  end-page: 7166
  ident: CR27
  article-title: Selective activation of ATF6 and PERK endoplasmic reticulum stress signaling pathways prevent mutant rhodopsin accumulation
  publication-title: Invest. Ophthalmol. Vis. Sci.
  doi: 10.1167/iovs.12-10222
– volume: 13
  start-page: 351
  year: 2007
  end-page: 364
  ident: CR41
  article-title: ATF6alpha optimizes long-term endoplasmic reticulum function to protect cells from chronic stress
  publication-title: Dev. Cell
  doi: 10.1016/j.devcel.2007.07.005
– volume: 16
  start-page: 653
  year: 2004
  end-page: 662
  ident: CR36
  article-title: Misfolded proteins, endoplasmic reticulum stress and neurodegeneration
  publication-title: Curr. Opin. Cell. Biol.
  doi: 10.1016/j.ceb.2004.09.012
– year: 2018
  ident: CR70
  article-title: Pharmacologic ATF6 activating compounds are metabolically activated to selectively modify endoplasmic reticulum proteins
  publication-title: Elife
  doi: 10.7554/eLife.37168
– volume: 56
  start-page: 6961
  year: 2015
  end-page: 6970
  ident: CR26
  article-title: In vivo visualization of endoplasmic reticulum stress in the retina using the ERAI reporter mouse
  publication-title: Invest. Ophthalmol. Vis. Sci.
  doi: 10.1167/iovs.15-16969
– volume: 3
  start-page: 1279
  year: 2013
  end-page: 1292
  ident: CR13
  article-title: Stress-independent activation of XBP1s and/or ATF6 reveals three functionally diverse ER proteostasis environments
  publication-title: Cell. Rep.
  doi: 10.1016/j.celrep.2013.03.024
– volume: 25
  start-page: 1210
  year: 2017
  end-page: 1216
  ident: CR46
  article-title: Autosomal recessive cone-rod dystrophy can be caused by mutations in the ATF6 gene
  publication-title: Eur. J. Hum. Genet.
  doi: 10.1038/ejhg.2017.131
– year: 2016
  ident: CR65
  article-title: Ceapins inhibit ATF6alpha signaling by selectively preventing transport of ATF6alpha to the Golgi apparatus during ER stress
  publication-title: Elife
  doi: 10.7554/eLife.11880
– volume: 29
  start-page: 335
  year: 2010
  end-page: 375
  ident: CR3
  article-title: The molecular basis of human retinal and vitreoretinal diseases
  publication-title: Prog. Retina Eye Res.
  doi: 10.1016/j.preteyeres.2010.03.004
– volume: 286
  start-page: 10551
  year: 2011
  end-page: 10567
  ident: CR23
  article-title: Probing mechanisms of photoreceptor degeneration in a new mouse model of the common form of autosomal dominant retinitis pigmentosa due to P23H opsin mutations
  publication-title: J. Biol. Chem.
  doi: 10.1074/jbc.M110.209759
– volume: 115
  start-page: 2907
  year: 2002
  end-page: 2918
  ident: CR25
  article-title: The cellular fate of mutant rhodopsin: quality control, degradation and aggresome formation
  publication-title: J. Cell Sci.
  doi: 10.1242/jcs.115.14.2907
– volume: 115
  start-page: 268
  year: 2005
  end-page: 281
  ident: CR29
  article-title: The unfolded protein response sensor IRE1alpha is required at 2 distinct steps in B cell lymphopoiesis
  publication-title: J. Clin. Invest.
  doi: 10.1172/JCI21848
– volume: 107
  start-page: 881
  year: 2001
  end-page: 891
  ident: CR11
  article-title: XBP1 mRNA is induced by ATF6 and spliced by IRE1 in response to ER stress to produce a highly active transcription factor
  publication-title: Cell
  doi: 10.1016/s0092-8674(01)00611-0
– volume: 33
  start-page: 2492
  year: 2014
  end-page: 2506
  ident: CR19
  article-title: Signal peptide peptidase functions in ERAD to cleave the unfolded protein response regulator XBP1u
  publication-title: EMBO J.
  doi: 10.15252/embj.201488208
– volume: 107
  start-page: 5961
  year: 2010
  end-page: 5966
  ident: CR42
  article-title: Restoration of visual function in P23H rhodopsin transgenic rats by gene delivery of BiP/Grp78
  publication-title: Proc. Natl. Acad. Sci. USA
  doi: 10.1073/pnas.0911991107
– volume: 4
  start-page: a013177
  year: 2012
  ident: CR15
  article-title: Protein-folding homeostasis in the endoplasmic reticulum and nutritional regulation
  publication-title: Cold Spring Harb. Perspect. Biol.
  doi: 10.1101/cshperspect.a013177
– volume: 42
  start-page: 589
  year: 2001
  end-page: 600
  ident: CR34
  article-title: The relationship between opsin overexpression and photoreceptor degeneration
  publication-title: Invest. Ophthalmol. Vis. Sci.
– volume: 11
  start-page: eaan5785
  year: 2018
  ident: CR48
  article-title: The unfolded protein response regulator ATF6 promotes mesodermal differentiation
  publication-title: Sci. Signal.
  doi: 10.1126/scisignal.aan5785
– volume: 10
  start-page: 547
  year: 2019
  ident: CR56
  article-title: Shifting the balance of autophagy and proteasome activation reduces proteotoxic cell death: A novel therapeutic approach for restoring photoreceptor homeostasis
  publication-title: Cell Death Dis.
  doi: 10.1038/s41419-019-1780-1
– volume: 88
  start-page: 6481
  year: 1991
  end-page: 6485
  ident: CR6
  article-title: Rhodopsin mutations in autosomal dominant retinitis pigmentosa
  publication-title: Proc. Natl. Acad. Sci. U S A
  doi: 10.1073/pnas.88.15.6481
– volume: 33
  start-page: 75
  year: 2008
  end-page: 89
  ident: CR40
  article-title: ATF6 is a transcription factor specializing in the regulation of quality control proteins in the endoplasmic reticulum
  publication-title: Cell Struct. Funct.
  doi: 10.1247/csf.07044
– volume: 23
  start-page: 758
  year: 2012
  ident: 95895_CR39
  publication-title: Mol. Biol. Cell
  doi: 10.1091/mbc.E11-08-0663
– volume: 114
  start-page: 400
  year: 2017
  ident: 95895_CR44
  publication-title: Proc. Natl. Acad. Sci. USA
  doi: 10.1073/pnas.1606387114
– year: 2021
  ident: 95895_CR73
  publication-title: Dev. Dyn.
  doi: 10.1002/dvdy.389
– volume: 56
  start-page: 3889
  year: 2015
  ident: 95895_CR47
  publication-title: Invest. Ophthalmol. Vis. Sci.
  doi: 10.1167/iovs.15-16778
– volume: 4
  start-page: a013177
  year: 2012
  ident: 95895_CR15
  publication-title: Cold Spring Harb. Perspect. Biol.
  doi: 10.1101/cshperspect.a013177
– year: 2016
  ident: 95895_CR67
  publication-title: Elife
  doi: 10.7554/eLife.15550
– volume: 115
  start-page: 2907
  year: 2002
  ident: 95895_CR25
  publication-title: J. Cell Sci.
  doi: 10.1242/jcs.115.14.2907
– volume: 66
  start-page: 157
  year: 2018
  ident: 95895_CR4
  publication-title: Prog. Retina Eye Res.
  doi: 10.1016/j.preteyeres.2018.03.005
– volume: 56
  start-page: 6961
  year: 2015
  ident: 95895_CR26
  publication-title: Invest. Ophthalmol. Vis. Sci.
  doi: 10.1167/iovs.15-16969
– volume: 14
  start-page: 1226
  year: 2018
  ident: 95895_CR55
  publication-title: Autophagy
  doi: 10.1080/15548627.2018.1463121
– year: 2016
  ident: 95895_CR64
  publication-title: Elife
  doi: 10.7554/eLife.11878
– volume: 8
  start-page: 191
  year: 2013
  ident: 95895_CR60
  publication-title: J. Ophthalmic Vis. Res.
– volume: 8
  start-page: 519
  year: 2007
  ident: 95895_CR16
  publication-title: Nat. Rev. Mol. Cell. Biol.
  doi: 10.1038/nrm2199
– volume: 33
  start-page: 75
  year: 2008
  ident: 95895_CR40
  publication-title: Cell Struct. Funct.
  doi: 10.1247/csf.07044
– volume: 9
  year: 2014
  ident: 95895_CR54
  publication-title: PLoS ONE
  doi: 10.1371/journal.pone.0083871
– volume: 21
  start-page: 1281
  year: 2015
  ident: 95895_CR74
  publication-title: Mol. Vis.
– volume: 286
  start-page: 10551
  year: 2011
  ident: 95895_CR23
  publication-title: J. Biol. Chem.
  doi: 10.1074/jbc.M110.209759
– volume: 14
  start-page: 152
  year: 2000
  ident: 95895_CR28
  publication-title: Genes Dev.
  doi: 10.1101/gad.14.2.152
– volume: 3
  start-page: 1279
  year: 2013
  ident: 95895_CR13
  publication-title: Cell. Rep.
  doi: 10.1016/j.celrep.2013.03.024
– volume: 10
  start-page: 547
  year: 2019
  ident: 95895_CR56
  publication-title: Cell Death Dis.
  doi: 10.1038/s41419-019-1780-1
– volume: 53
  start-page: 7159
  year: 2012
  ident: 95895_CR27
  publication-title: Invest. Ophthalmol. Vis. Sci.
  doi: 10.1167/iovs.12-10222
– volume: 213
  start-page: 809
  year: 2007
  ident: 95895_CR59
  publication-title: J. Cell Physiol.
  doi: 10.1002/jcp.21152
– volume: 9
  start-page: 815
  year: 1992
  ident: 95895_CR33
  publication-title: Neuron
  doi: 10.1016/0896-6273(92)90236-7
– volume: 47
  start-page: 757
  year: 2015
  ident: 95895_CR30
  publication-title: Nat. Genet.
  doi: 10.1038/ng.3319
– volume: 18
  start-page: 1133
  year: 2004
  ident: 95895_CR62
  publication-title: Eye (Lond)
  doi: 10.1038/sj.eye.6701573
– volume: 277
  start-page: 34150
  year: 2002
  ident: 95895_CR37
  publication-title: J. Biol. Chem.
  doi: 10.1074/jbc.M204955200
– volume: 34
  start-page: 1659
  year: 1993
  ident: 95895_CR1
  publication-title: Invest. Ophthalmol. Vis. Sci.
– volume: 88
  start-page: 6481
  year: 1991
  ident: 95895_CR6
  publication-title: Proc. Natl. Acad. Sci. U S A
  doi: 10.1073/pnas.88.15.6481
– volume: 6
  start-page: 1355
  year: 2000
  ident: 95895_CR21
  publication-title: Mol. Cell
  doi: 10.1016/s1097-2765(00)00133-7
– volume: 368
  start-page: 1795
  year: 2006
  ident: 95895_CR2
  publication-title: Lancet
  doi: 10.1016/S0140-6736(06)69740-7
– volume: 334
  start-page: 1081
  year: 2011
  ident: 95895_CR8
  publication-title: Science
  doi: 10.1126/science.1209038
– volume: 854
  start-page: 185
  year: 2016
  ident: 95895_CR31
  publication-title: Adv. Exp. Med. Biol.
  doi: 10.1007/978-3-319-17121-0_25
– volume: 323
  start-page: 1302
  year: 1990
  ident: 95895_CR5
  publication-title: N. Engl. J. Med.
  doi: 10.1056/NEJM199011083231903
– volume: 33
  start-page: 6121
  year: 1994
  ident: 95895_CR38
  publication-title: Biochemistry
  doi: 10.1021/bi00186a011
– volume: 415
  start-page: 92
  year: 2002
  ident: 95895_CR9
  publication-title: Nature
  doi: 10.1038/415092a
– volume: 56
  start-page: 169
  year: 2016
  ident: 95895_CR61
  publication-title: Ophthalmic Res.
  doi: 10.1159/000446321
– year: 2016
  ident: 95895_CR65
  publication-title: Elife
  doi: 10.7554/eLife.11880
– volume: 182
  start-page: 1412
  year: 2013
  ident: 95895_CR49
  publication-title: Am. J. Pathol.
  doi: 10.1016/j.ajpath.2012.12.020
– volume: 134
  start-page: 941
  year: 2015
  ident: 95895_CR43
  publication-title: Hum. Genet.
  doi: 10.1007/s00439-015-1571-4
– volume: 11
  start-page: eaan5785
  year: 2018
  ident: 95895_CR48
  publication-title: Sci. Signal.
  doi: 10.1126/scisignal.aan5785
– volume: 23
  start-page: 7448
  year: 2003
  ident: 95895_CR12
  publication-title: Mol. Cell. Biol.
  doi: 10.1128/mcb.23.21.7448-7459.2003
– ident: 95895_CR50
– volume: 723
  start-page: 559
  year: 2012
  ident: 95895_CR53
  publication-title: Adv. Exp. Med. Biol.
  doi: 10.1007/978-1-4614-0631-0_71
– volume: 520
  start-page: 874
  year: 2012
  ident: 95895_CR71
  publication-title: J. Comp. Neurol.
  doi: 10.1002/cne.22800
– volume: 10
  start-page: 187
  year: 2019
  ident: 95895_CR63
  publication-title: Nat. Commun.
  doi: 10.1038/s41467-018-08129-2
– volume: 52
  start-page: 679
  year: 2015
  ident: 95895_CR22
  publication-title: Mol. Neurobiol.
  doi: 10.1007/s12035-014-8881-8
– volume: 62
  start-page: 3073
  year: 2021
  ident: 95895_CR51
  publication-title: IOVS
– volume: 91
  start-page: 974
  year: 1994
  ident: 95895_CR58
  publication-title: Proc. Natl. Acad. Sci. USA
  doi: 10.1073/pnas.91.3.974
– volume: 16
  start-page: 653
  year: 2004
  ident: 95895_CR36
  publication-title: Curr. Opin. Cell. Biol.
  doi: 10.1016/j.ceb.2004.09.012
– volume: 107
  start-page: 5961
  year: 2010
  ident: 95895_CR42
  publication-title: Proc. Natl. Acad. Sci. USA
  doi: 10.1073/pnas.0911991107
– volume: 22
  start-page: 133
  year: 2001
  ident: 95895_CR7
  publication-title: Ophthalmic Genet.
  doi: 10.1076/opge.22.3.133.2224
– volume: 79
  start-page: 719
  year: 2001
  ident: 95895_CR35
  publication-title: J. Neurochem.
  doi: 10.1046/j.1471-4159.2001.00623.x
– volume: 167
  start-page: 56
  year: 2018
  ident: 95895_CR32
  publication-title: Exp. Eye Res.
  doi: 10.1016/j.exer.2017.10.023
– volume: 98
  start-page: 1186
  year: 2006
  ident: 95895_CR66
  publication-title: Circ. Res.
  doi: 10.1161/01.RES.0000220643.65941.8d
– volume: 25
  start-page: 1210
  year: 2017
  ident: 95895_CR46
  publication-title: Eur. J. Hum. Genet.
  doi: 10.1038/ejhg.2017.131
– volume: 295
  start-page: 15692
  year: 2020
  ident: 95895_CR69
  publication-title: J. Biol. Chem.
  doi: 10.1074/jbc.REV120.010218
– year: 2019
  ident: 95895_CR68
  publication-title: Elife
  doi: 10.7554/eLife.46595
– volume: 10
  start-page: 3787
  year: 1999
  ident: 95895_CR20
  publication-title: Mol. Biol. Cell
  doi: 10.1091/mbc.10.11.3787
– volume: 53
  start-page: 5527
  year: 2016
  ident: 95895_CR72
  publication-title: Mol. Neurobiol.
  doi: 10.1007/s12035-015-9456-z
– volume: 115
  start-page: 268
  year: 2005
  ident: 95895_CR29
  publication-title: J. Clin. Invest.
  doi: 10.1172/JCI21848
– year: 2018
  ident: 95895_CR70
  publication-title: Elife
  doi: 10.7554/eLife.37168
– volume: 107
  start-page: 881
  year: 2001
  ident: 95895_CR11
  publication-title: Cell
  doi: 10.1016/s0092-8674(01)00611-0
– volume: 397
  start-page: 271
  year: 1999
  ident: 95895_CR14
  publication-title: Nature
  doi: 10.1038/16729
– volume: 279
  start-page: 46580
  year: 2004
  ident: 95895_CR57
  publication-title: J. Biol. Chem.
  doi: 10.1074/jbc.M406685200
– volume: 42
  start-page: 589
  year: 2001
  ident: 95895_CR34
  publication-title: Invest. Ophthalmol. Vis. Sci.
– year: 2020
  ident: 95895_CR45
  publication-title: JCI Insight
  doi: 10.1172/jci.insight.136041
– volume: 13
  start-page: 184
  year: 2011
  ident: 95895_CR18
  publication-title: Nat. Cell. Biol.
  doi: 10.1038/ncb0311-184
– volume: 23
  start-page: 1723
  year: 2014
  ident: 95895_CR24
  publication-title: Hum. Mol. Genet.
  doi: 10.1093/hmg/ddt561
– volume: 33
  start-page: 2492
  year: 2014
  ident: 95895_CR19
  publication-title: EMBO J.
  doi: 10.15252/embj.201488208
– volume: 73
  start-page: 1197
  year: 1993
  ident: 95895_CR10
  publication-title: Cell
  doi: 10.1016/0092-8674(93)90648-a
– volume: 13
  start-page: 351
  year: 2007
  ident: 95895_CR41
  publication-title: Dev. Cell
  doi: 10.1016/j.devcel.2007.07.005
– volume: 16
  year: 2020
  ident: 95895_CR52
  publication-title: PLoS Genet.
  doi: 10.1371/journal.pgen.1009172
– volume: 56
  start-page: 911
  year: 2013
  ident: 95895_CR17
  publication-title: Diabetologia
  doi: 10.1007/s00125-012-2809-5
– volume: 29
  start-page: 335
  year: 2010
  ident: 95895_CR3
  publication-title: Prog. Retina Eye Res.
  doi: 10.1016/j.preteyeres.2010.03.004
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Snippet Retinitis Pigmentosa (RP) is a blinding disease that arises from loss of rods and subsequently cones. The P23H rhodopsin knock-in (P23H-KI) mouse develops...
Abstract Retinitis Pigmentosa (RP) is a blinding disease that arises from loss of rods and subsequently cones. The P23H rhodopsin knock-in (P23H-KI) mouse...
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StartPage 16356
SubjectTerms 631/45
692/4017
692/420
Activating transcription factor 6
Activating Transcription Factor 6 - metabolism
Animals
Cones
Disease Models, Animal
Female
Homeostasis
Homeostasis - physiology
Humanities and Social Sciences
Male
Mice
Mice, Inbred C57BL
multidisciplinary
Phenotypes
Photoreceptors
Protein folding
Proteins
Retina
Retina - metabolism
Retinal degeneration
Retinal Degeneration - metabolism
Retinal Rod Photoreceptor Cells - metabolism
Retinitis
Retinitis pigmentosa
Retinitis Pigmentosa - metabolism
Rhodopsin
Rhodopsin - metabolism
Science
Science (multidisciplinary)
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Title ATF6 is required for efficient rhodopsin clearance and retinal homeostasis in the P23H rho retinitis pigmentosa mouse model
URI https://link.springer.com/article/10.1038/s41598-021-95895-7
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