Photoreceptor-induced RPE phagolysosomal maturation defects in Stargardt-like Maculopathy (STGD3)
For many neurodegenerative disorders, expression of a pathological protein by one cell type impedes function of other cell types, which in turn contributes to the death of the first cell type. In transgenic mice modelling Stargardt-like (STGD3) maculopathy, human mutant ELOVL4 expression by photorec...
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Published in | Scientific reports Vol. 8; no. 1; pp. 5944 - 14 |
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Main Authors | , , , , , |
Format | Journal Article |
Language | English |
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Nature Publishing Group UK
13.04.2018
Nature Publishing Group Nature Portfolio |
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Abstract | For many neurodegenerative disorders, expression of a pathological protein by one cell type impedes function of other cell types, which in turn contributes to the death of the first cell type. In transgenic mice modelling Stargardt-like (STGD3) maculopathy, human mutant ELOVL4 expression by photoreceptors is associated with defects in the underlying retinal pigment epithelium (RPE). To examine how photoreceptors exert cytotoxic effects on RPE cells, transgenic ELOVL4 (TG1–2 line; TG) and wild-type (WT) littermates were studied one month prior (preclinical stage) to onset of photoreceptor loss (two months). TG photoreceptor outer segments presented to human RPE cells are recognized and internalized into phagosomes, but their digestion is delayed. Live RPE cell imaging pinpoints decreased numbers of acidified phagolysomes.
In vivo
, master regulator of lysosomal genes, transcription factor EB (TFEB), and key lysosomal enzyme Cathepsin D are both unaffected. Oxidative stress, as ruled out with high-resolution respirometry, does not play a role at such an early stage. Upregulation of CRYBA1/A3 and phagocytic cells (microglia/macrophages) interposed between RPE and photoreceptors support adaptive responses to processing delays. Impaired phagolysosomal maturation is observed in RPE of mice expressing human mutant ELOVL4 in their photoreceptors prior to photoreceptor death and associated vision loss. |
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AbstractList | Abstract For many neurodegenerative disorders, expression of a pathological protein by one cell type impedes function of other cell types, which in turn contributes to the death of the first cell type. In transgenic mice modelling Stargardt-like (STGD3) maculopathy, human mutant ELOVL4 expression by photoreceptors is associated with defects in the underlying retinal pigment epithelium (RPE). To examine how photoreceptors exert cytotoxic effects on RPE cells, transgenic ELOVL4 (TG1–2 line; TG) and wild-type (WT) littermates were studied one month prior (preclinical stage) to onset of photoreceptor loss (two months). TG photoreceptor outer segments presented to human RPE cells are recognized and internalized into phagosomes, but their digestion is delayed. Live RPE cell imaging pinpoints decreased numbers of acidified phagolysomes. In vivo, master regulator of lysosomal genes, transcription factor EB (TFEB), and key lysosomal enzyme Cathepsin D are both unaffected. Oxidative stress, as ruled out with high-resolution respirometry, does not play a role at such an early stage. Upregulation of CRYBA1/A3 and phagocytic cells (microglia/macrophages) interposed between RPE and photoreceptors support adaptive responses to processing delays. Impaired phagolysosomal maturation is observed in RPE of mice expressing human mutant ELOVL4 in their photoreceptors prior to photoreceptor death and associated vision loss. Abstract For many neurodegenerative disorders, expression of a pathological protein by one cell type impedes function of other cell types, which in turn contributes to the death of the first cell type. In transgenic mice modelling Stargardt-like (STGD3) maculopathy, human mutant ELOVL4 expression by photoreceptors is associated with defects in the underlying retinal pigment epithelium (RPE). To examine how photoreceptors exert cytotoxic effects on RPE cells, transgenic ELOVL4 (TG1–2 line; TG) and wild-type (WT) littermates were studied one month prior (preclinical stage) to onset of photoreceptor loss (two months). TG photoreceptor outer segments presented to human RPE cells are recognized and internalized into phagosomes, but their digestion is delayed. Live RPE cell imaging pinpoints decreased numbers of acidified phagolysomes. In vivo , master regulator of lysosomal genes, transcription factor EB (TFEB), and key lysosomal enzyme Cathepsin D are both unaffected. Oxidative stress, as ruled out with high-resolution respirometry, does not play a role at such an early stage. Upregulation of CRYBA1/A3 and phagocytic cells (microglia/macrophages) interposed between RPE and photoreceptors support adaptive responses to processing delays. Impaired phagolysosomal maturation is observed in RPE of mice expressing human mutant ELOVL4 in their photoreceptors prior to photoreceptor death and associated vision loss. For many neurodegenerative disorders, expression of a pathological protein by one cell type impedes function of other cell types, which in turn contributes to the death of the first cell type. In transgenic mice modelling Stargardt-like (STGD3) maculopathy, human mutant ELOVL4 expression by photoreceptors is associated with defects in the underlying retinal pigment epithelium (RPE). To examine how photoreceptors exert cytotoxic effects on RPE cells, transgenic ELOVL4 (TG1–2 line; TG) and wild-type (WT) littermates were studied one month prior (preclinical stage) to onset of photoreceptor loss (two months). TG photoreceptor outer segments presented to human RPE cells are recognized and internalized into phagosomes, but their digestion is delayed. Live RPE cell imaging pinpoints decreased numbers of acidified phagolysomes. In vivo , master regulator of lysosomal genes, transcription factor EB (TFEB), and key lysosomal enzyme Cathepsin D are both unaffected. Oxidative stress, as ruled out with high-resolution respirometry, does not play a role at such an early stage. Upregulation of CRYBA1/A3 and phagocytic cells (microglia/macrophages) interposed between RPE and photoreceptors support adaptive responses to processing delays. Impaired phagolysosomal maturation is observed in RPE of mice expressing human mutant ELOVL4 in their photoreceptors prior to photoreceptor death and associated vision loss. For many neurodegenerative disorders, expression of a pathological protein by one cell type impedes function of other cell types, which in turn contributes to the death of the first cell type. In transgenic mice modelling Stargardt-like (STGD3) maculopathy, human mutant ELOVL4 expression by photoreceptors is associated with defects in the underlying retinal pigment epithelium (RPE). To examine how photoreceptors exert cytotoxic effects on RPE cells, transgenic ELOVL4 (TG1-2 line; TG) and wild-type (WT) littermates were studied one month prior (preclinical stage) to onset of photoreceptor loss (two months). TG photoreceptor outer segments presented to human RPE cells are recognized and internalized into phagosomes, but their digestion is delayed. Live RPE cell imaging pinpoints decreased numbers of acidified phagolysomes. In vivo, master regulator of lysosomal genes, transcription factor EB (TFEB), and key lysosomal enzyme Cathepsin D are both unaffected. Oxidative stress, as ruled out with high-resolution respirometry, does not play a role at such an early stage. Upregulation of CRYBA1/A3 and phagocytic cells (microglia/macrophages) interposed between RPE and photoreceptors support adaptive responses to processing delays. Impaired phagolysosomal maturation is observed in RPE of mice expressing human mutant ELOVL4 in their photoreceptors prior to photoreceptor death and associated vision loss. |
ArticleNumber | 5944 |
Author | Sauvé, Yves Dejos, Camille Han, Woo Hyun Lemieux, Hélène Kuny, Sharee Capel, Heather |
Author_xml | – sequence: 1 givenname: Camille surname: Dejos fullname: Dejos, Camille organization: Department of Ophthalmology and Visual Sciences, University of Alberta – sequence: 2 givenname: Sharee surname: Kuny fullname: Kuny, Sharee organization: Department of Ophthalmology and Visual Sciences, University of Alberta – sequence: 3 givenname: Woo Hyun surname: Han fullname: Han, Woo Hyun organization: Department of Ophthalmology and Visual Sciences, University of Alberta – sequence: 4 givenname: Heather surname: Capel fullname: Capel, Heather organization: Department of Physiology, University of Alberta – sequence: 5 givenname: Hélène surname: Lemieux fullname: Lemieux, Hélène organization: Faculty Saint-Jean, University of Alberta – sequence: 6 givenname: Yves surname: Sauvé fullname: Sauvé, Yves email: ysauve@ualberta.ca organization: Department of Ophthalmology and Visual Sciences, University of Alberta, Department of Physiology, University of Alberta |
BackLink | https://www.ncbi.nlm.nih.gov/pubmed/29654292$$D View this record in MEDLINE/PubMed |
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Cites_doi | 10.1167/iovs.11-8418 10.1073/pnas.0504759102 10.1073/pnas.1214707110 10.1074/jbc.M115.687004 10.1016/j.exer.2010.11.008 10.1167/iovs.13-13099 10.1126/science.1204592 10.1016/j.preteyeres.2011.12.001 10.1016/j.preteyeres.2009.06.001 10.1242/jcs.078790 10.1126/science.982063 10.1189/jlb.0207114 10.1371/journal.pone.0007945 10.1073/pnas.222551899 10.1167/iovs.15-17567 10.1167/iovs.09-4718 10.1126/science.282.5386.117 10.1111/cns.12655 10.1167/iovs.17-21929 10.1007/978-1-62703-080-9_20 10.1016/S2214-109X(13)70145-1 10.1074/jbc.M112.448712 10.1073/pnas.0407698102 10.1038/emboj.2012.32 10.1111/j.1755-3768.2011.02179.x 10.1074/jbc.M503411200 10.1038/nprot.2016.136 10.1073/pnas.0605818104 10.1096/fj.03-0289fje 10.1167/iovs.14-13995 10.1073/pnas.0802607105 10.1111/acel.12561 10.1007/978-3-319-17121-0_95 10.1016/j.exer.2015.09.015 10.1167/iovs.02-0438 10.1083/jcb.42.2.392 10.1016/j.exer.2009.07.021 10.3389/fnana.2017.00014 10.1016/j.neurobiolaging.2005.05.012 10.1167/iovs.04-1510 10.1016/j.exer.2014.01.010 10.1074/jbc.M116.770784 10.1073/pnas.1217251110 10.2353/ajpath.2009.090023 10.1126/science.1174447 10.1242/jeb.201.8.1129 |
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References | Sardiello (CR21) 2009; 325 Logan (CR5) 2013; 110 Xu, Toops, Lakkaraju (CR20) 2013; 54 Karan (CR29) 2005; 102 LaVail, Rod (CR45) 1976; 194 Golpich (CR41) 2017; 23 Agbaga (CR1) 2008; 105 Crabb (CR26) 2002; 99 Duncan (CR12) 2003; 44 Barabas (CR3) 2013; 110 Samuel (CR46) 2017; 23 Mao, Finnemann (CR47) 2013; 935 Settembre (CR23) 2011; 332 Sethna (CR31) 2016; 291 Bennett (CR6) 2014; 55 Grayson, Molday (CR2) 2005; 280 Ma, Zhao, Fontainhas, Fariss, Wong (CR39) 2009; 4 Vasireddy (CR34) 2009; 89 Di Pierdomenico (CR44) 2017; 11 Feher (CR42) 2006; 27 Young, Bok (CR11) 1969; 42 Shang (CR35) 2017; 16 Mazzoni, Safa, Finnemann (CR48) 2014; 126 Xu, Chen, Forrester (CR38) 2009; 28 Kuny, Cho, Dimopoulos, Sauvé (CR8) 2015; 56 Mao, Finnemann (CR19) 2016; 854 Agbaga (CR7) 2014; 55 Zigler (CR15) 2011; 124 Yaung (CR25) 2007; 13 Gnaiger, Lassnig, Kuznetsov, Rieger, Margreiter (CR27) 1998; 201 Joly (CR37) 2009; 174 Sparrow (CR9) 2012; 31 Tian (CR24) 2005; 102 Krock, Bilotta, Perkins (CR10) 2007; 104 Tsang (CR49) 1998; 282 Reyes-Reveles (CR43) 2017; 292 Langmann (CR30) 2007; 81 Settembre (CR22) 2012; 31 Mata (CR32) 2001; 42 Kuny (CR18) 2010; 51 Han (CR28) 2017; 58 Kohno (CR40) 2013; 288 Fort, Lampi (CR36) 2011; 92 Kinnunen, Petrovski, Moe, Berta, Kaarniranta (CR16) 2012; 90 Tyanova, Temu, Cox (CR51) 2016; 11 Wong (CR17) 2014; 2 Zhang (CR14) 2005; 46 Kuny, Gaillard, Sauvé (CR4) 2012; 53 Wei, Xun, Granado, Wu, Handa (CR50) 2016; 145 Bergmann, Schütt, Holz, Kopitz (CR33) 2004; 18 Picard (CR13) 2010; 2 BL Krock (24357_CR10) 2007; 104 RW Young (24357_CR11) 1969; 42 JS Zigler (24357_CR15) 2011; 124 M Sardiello (24357_CR21) 2009; 325 K Kinnunen (24357_CR16) 2012; 90 Y Mao (24357_CR47) 2013; 935 V Vasireddy (24357_CR34) 2009; 89 D Zhang (24357_CR14) 2005; 46 J Xu (24357_CR20) 2013; 54 JW Crabb (24357_CR26) 2002; 99 F Mazzoni (24357_CR48) 2014; 126 C Grayson (24357_CR2) 2005; 280 H Xu (24357_CR38) 2009; 28 T Langmann (24357_CR30) 2007; 81 W Ma (24357_CR39) 2009; 4 Y Mao (24357_CR19) 2016; 854 JR Sparrow (24357_CR9) 2012; 31 W Samuel (24357_CR46) 2017; 23 MP Agbaga (24357_CR7) 2014; 55 LD Bennett (24357_CR6) 2014; 55 G Karan (24357_CR29) 2005; 102 P Barabas (24357_CR3) 2013; 110 M Bergmann (24357_CR33) 2004; 18 S Joly (24357_CR37) 2009; 174 C Settembre (24357_CR22) 2012; 31 S Logan (24357_CR5) 2013; 110 MP Agbaga (24357_CR1) 2008; 105 E Picard (24357_CR13) 2010; 2 M Golpich (24357_CR41) 2017; 23 H Wei (24357_CR50) 2016; 145 J Feher (24357_CR42) 2006; 27 P Shang (24357_CR35) 2017; 16 S Kuny (24357_CR4) 2012; 53 J Yaung (24357_CR25) 2007; 13 MM LaVail (24357_CR45) 1976; 194 J Reyes-Reveles (24357_CR43) 2017; 292 JL Duncan (24357_CR12) 2003; 44 J Di Pierdomenico (24357_CR44) 2017; 11 S Kuny (24357_CR18) 2010; 51 NL Mata (24357_CR32) 2001; 42 PE Fort (24357_CR36) 2011; 92 WL Wong (24357_CR17) 2014; 2 SH Tsang (24357_CR49) 1998; 282 S Sethna (24357_CR31) 2016; 291 C Settembre (24357_CR23) 2011; 332 E Gnaiger (24357_CR27) 1998; 201 S Kuny (24357_CR8) 2015; 56 J Tian (24357_CR24) 2005; 102 S Tyanova (24357_CR51) 2016; 11 WH Han (24357_CR28) 2017; 58 H Kohno (24357_CR40) 2013; 288 |
References_xml | – volume: 53 start-page: 664 year: 2012 end-page: 675 ident: CR4 article-title: Differential gene expression in eyecup and retina of a mouse model of Stargardt-like macular dystrophy (STGD3) publication-title: Invest. Ophthalmol. Vis. Sci. doi: 10.1167/iovs.11-8418 contributor: fullname: Sauvé – volume: 102 start-page: 11846 year: 2005 end-page: 11851 ident: CR24 article-title: Advanced glycation endproduct-induced aging of the retinal pigment epithelium and choroid: a comprehensive transcriptional response publication-title: Proc. Natl. Acad. Sci. USA doi: 10.1073/pnas.0504759102 contributor: fullname: Tian – volume: 110 start-page: 5181 year: 2013 end-page: 5186 ident: CR3 article-title: Role of ELOVL4 and very long-chain polyunsaturated fatty acids in mouse models of Stargardt type 3 retinal degeneration publication-title: Proc. Natl. Acad. Sci. USA doi: 10.1073/pnas.1214707110 contributor: fullname: Barabas – volume: 291 start-page: 6494 year: 2016 end-page: 6506 ident: CR31 article-title: Regulation of Phagolysosomal Digestion by Caveolin-1 of the Retinal Pigment Epithelium Is Essential for Vision publication-title: J. Biol. Chem. doi: 10.1074/jbc.M115.687004 contributor: fullname: Sethna – volume: 92 start-page: 98 year: 2011 end-page: 103 ident: CR36 article-title: New focus on alpha-crystallins in retinal neurodegenerative diseases publication-title: Exp. Eye Res. doi: 10.1016/j.exer.2010.11.008 contributor: fullname: Lampi – volume: 55 start-page: 3669 year: 2014 end-page: 3680 ident: CR7 article-title: Mutant ELOVL4 that causes autosomal dominant stargardt-3 macular dystrophy is misrouted to rod OS disks publication-title: Invest. Ophthalmol. Vis. Sci. doi: 10.1167/iovs.13-13099 contributor: fullname: Agbaga – volume: 2 start-page: 981 year: 2010 end-page: 989 ident: CR13 article-title: CD36 plays an important role in the clearance of oxLDL and associated age-dependent sub-retinal deposits publication-title: Aging (Albany N.Y.) contributor: fullname: Picard – volume: 332 start-page: 1429 year: 2011 end-page: 1433 ident: CR23 article-title: TFEB links autophagy to lysosomal biogenesis publication-title: Science doi: 10.1126/science.1204592 contributor: fullname: Settembre – volume: 31 start-page: 121 year: 2012 end-page: 135 ident: CR9 article-title: The bisretinoids of retinal pigment epithelium publication-title: Prog. Retin. Eye Res. doi: 10.1016/j.preteyeres.2011.12.001 contributor: fullname: Sparrow – volume: 28 start-page: 348 year: 2009 end-page: 368 ident: CR38 article-title: Para-inflammation in the aging retina publication-title: Prog. Retin. Eye Res. doi: 10.1016/j.preteyeres.2009.06.001 contributor: fullname: Forrester – volume: 124 start-page: 523 year: 2011 end-page: 531 ident: CR15 article-title: Mutation in the βA3/A1-crystallin gene impairs phagosome degradation in the retinal pigmented epithelium of the rat publication-title: J. Cell Sci. doi: 10.1242/jcs.078790 contributor: fullname: Zigler – volume: 194 start-page: 1071 year: 1976 end-page: 1074 ident: CR45 article-title: disk shedding in rat retina: relationship to cyclic lighting publication-title: Science doi: 10.1126/science.982063 contributor: fullname: Rod – volume: 81 start-page: 1345 year: 2007 end-page: 1351 ident: CR30 article-title: Microglia activation in retinal degeneration publication-title: J. Leukoc. Biol. doi: 10.1189/jlb.0207114 contributor: fullname: Langmann – volume: 4 start-page: e7945 year: 2009 ident: CR39 article-title: Microglia in the mouse retina alter the structure and function of retinal pigmented epithelial cells: a potential cellular interaction relevant to AMD publication-title: PLoS One. doi: 10.1371/journal.pone.0007945 contributor: fullname: Wong – volume: 99 start-page: 14682 year: 2002 end-page: 14687 ident: CR26 article-title: Drusen proteome analysis: an approach to the etiology of age-related macular degeneration publication-title: Proc. Natl. Acad. Sci. USA doi: 10.1073/pnas.222551899 contributor: fullname: Crabb – volume: 56 start-page: 7109 year: 2015 end-page: 7121 ident: CR8 article-title: Early Onset Ultrastructural and Functional Defects in RPE and Photoreceptors of a Stargardt-Like Macular Dystrophy (STGD3) Transgenic Mouse Model publication-title: Invest. Ophthalmol. Vis. Sci. doi: 10.1167/iovs.15-17567 contributor: fullname: Sauvé – volume: 51 start-page: 2248 year: 2010 end-page: 2262 ident: CR18 article-title: Inner retina remodeling in a mouse model of Stargardt-like macular dystrophy (STGD3) publication-title: Invest. Ophthalmol. Vis. Sci. doi: 10.1167/iovs.09-4718 contributor: fullname: Kuny – volume: 282 start-page: 117 year: 1998 end-page: 121 ident: CR49 article-title: Role for the target enzyme in deactivation of photoreceptor G protein publication-title: Science doi: 10.1126/science.282.5386.117 contributor: fullname: Tsang – volume: 42 start-page: 1685 year: 2001 end-page: 1690 ident: CR32 article-title: Delayed dark-adaptation and lipofuscin accumulation in abcr +/− mice: implications for involvement of ABCR in age-related macular degeneration publication-title: Invest. Ophthalmol. Vis. Sci. contributor: fullname: Mata – volume: 23 start-page: 5 year: 2017 end-page: 22 ident: CR41 article-title: Mitochondrial Dysfunction and Biogenesis in Neurodegenerative diseases: Pathogenesis and Treatment publication-title: CNS Neurosci. Ther. doi: 10.1111/cns.12655 contributor: fullname: Golpich – volume: 58 start-page: 3826 year: 2017 end-page: 3839 ident: CR28 article-title: Modifications in Retinal Mitochondrial Respiration Precede Type 2 Diabetes and Protracted Microvascular Retinopathy publication-title: Invest. Ophthalmol. Vis. Sci. doi: 10.1167/iovs.17-21929 contributor: fullname: Han – volume: 935 start-page: 285 year: 2013 end-page: 295 ident: CR47 article-title: Analysis of photoreceptor outer segment phagocytosis by RPE cells in culture publication-title: Methods Mol. Biol. doi: 10.1007/978-1-62703-080-9_20 contributor: fullname: Finnemann – volume: 2 start-page: e106 year: 2014 end-page: 116 ident: CR17 article-title: Global prevalence of age-related macular degeneration and disease burden projection for 2020 and 2040: a systematic review and meta-analysis publication-title: Lancet Glob. Health doi: 10.1016/S2214-109X(13)70145-1 contributor: fullname: Wong – volume: 288 start-page: 15326 year: 2013 end-page: 15341 ident: CR40 article-title: Photoreceptor proteins initiate microglial activation via Toll-like receptor 4 in retinal degeneration mediated by all-trans-retinal publication-title: J. Biol. Chem. doi: 10.1074/jbc.M112.448712 contributor: fullname: Kohno – volume: 102 start-page: 4164 year: 2005 end-page: 4169 ident: CR29 article-title: Lipofuscin accumulation, abnormal electrophysiology, and photoreceptor degeneration in mutant ELOVL4 transgenic mice: a model for macular degeneration publication-title: Proc. Natl. Acad. Sci. USA doi: 10.1073/pnas.0407698102 contributor: fullname: Karan – volume: 31 start-page: 1095 year: 2012 end-page: 1108 ident: CR22 article-title: A lysosome-to-nucleus signalling mechanism senses and regulates the lysosome via mTOR and TFEB publication-title: EMBO J. doi: 10.1038/emboj.2012.32 contributor: fullname: Settembre – volume: 90 start-page: 299 year: 2012 end-page: 309 ident: CR16 article-title: Molecular mechanisms of retinal pigment epithelium damage and development of age-related macular degeneration publication-title: Acta Ophthalmol. doi: 10.1111/j.1755-3768.2011.02179.x contributor: fullname: Kaarniranta – volume: 280 start-page: 32521 year: 2005 end-page: 32530 ident: CR2 article-title: Dominant negative mechanism underlies autosomal dominant Stargardt-like macular dystrophy linked to mutations in ELOVL4 publication-title: J. Biol. Chem. doi: 10.1074/jbc.M503411200 contributor: fullname: Molday – volume: 325 start-page: 473 year: 2009 end-page: 477 ident: CR21 article-title: A gene network regulating lysosomal biogenesis and function publication-title: Science contributor: fullname: Sardiello – volume: 11 start-page: 2301 year: 2016 end-page: 2319 ident: CR51 article-title: The MaxQuant computational platform for mass spectrometry-based shotgun proteomics publication-title: Nat.Protoc. doi: 10.1038/nprot.2016.136 contributor: fullname: Cox – volume: 104 start-page: 4600 year: 2007 end-page: 4605 ident: CR10 article-title: Non cell-autonomous photoreceptor degeneration in a zebrafish model of choroideremia publication-title: Proc. Natl. Acad. Sci. USA doi: 10.1073/pnas.0605818104 contributor: fullname: Perkins – volume: 13 start-page: 566 year: 2007 end-page: 577 ident: CR25 article-title: alpha-Crystallin distribution in retinal pigment epithelium and effect of gene knockouts on sensitivity to oxidative stress publication-title: Mol. Vis. contributor: fullname: Yaung – volume: 18 start-page: 562 year: 2004 end-page: 564 ident: CR33 article-title: Inhibition of the ATP-driven proton pump in RPE lysosomes by the major lipofuscin fluorophore A2-E may contribute to the pathogenesis of age-related macular degeneration publication-title: FASEB J. doi: 10.1096/fj.03-0289fje contributor: fullname: Kopitz – volume: 55 start-page: 3150 year: 2014 end-page: 3157 ident: CR6 article-title: Effect of reduced retinal VLC-PUFA on rod and cone photoreceptors publication-title: Invest. Ophthalmol. Vis. Sci. doi: 10.1167/iovs.14-13995 contributor: fullname: Bennett – volume: 105 start-page: 12843 year: 2008 end-page: 12848 ident: CR1 article-title: Role of Stargardt-3 macular dystrophy protein (ELOVL4) in the biosynthesis of very long chain fatty acids publication-title: Proc. Natl. Acad. Sci. USA doi: 10.1073/pnas.0802607105 contributor: fullname: Agbaga – volume: 201 start-page: 1129 year: 1998 end-page: 1139 ident: CR27 article-title: Mitochondrial oxygen affinity, respiratory flux control and excess capacity of cytochrome oxidase publication-title: J. Exp. Biol. contributor: fullname: Margreiter – volume: 16 start-page: 349 year: 2017 end-page: 359 ident: CR35 article-title: The amino acid transporter SLC36A4 regulates the amino acid pool in retinal pigmented epithelial cells and mediates the mechanistic target of rapamycin, complex 1 signaling publication-title: Aging Cell doi: 10.1111/acel.12561 contributor: fullname: Shang – volume: 854 start-page: 717 year: 2016 end-page: 723 ident: CR19 article-title: Live imaging of LysoTracker-labelled phagolysosomes tracks diurnal phagocytosis of photoreceptor outer segment fragments in rat RPE tissue publication-title: Adv. Exp. Med. Biol. doi: 10.1007/978-3-319-17121-0_95 contributor: fullname: Finnemann – volume: 145 start-page: 450 year: 2016 end-page: 455 ident: CR50 article-title: An easy, rapid method to isolate RPE cell protein from the mouse eye publication-title: Exp. Eye Res. doi: 10.1016/j.exer.2015.09.015 contributor: fullname: Handa – volume: 44 start-page: 826 year: 2003 end-page: 838 ident: CR12 article-title: An RCS-like retinal dystrophy phenotype in mer knockout mice publication-title: Invest. Ophthalmol. Vis. Sci. doi: 10.1167/iovs.02-0438 contributor: fullname: Duncan – volume: 42 start-page: 392 year: 1969 end-page: 403 ident: CR11 article-title: Participation of the retinal pigment epithelium in the rod outer segment renewal process publication-title: J. Cell Biol. doi: 10.1083/jcb.42.2.392 contributor: fullname: Bok – volume: 89 start-page: 905 year: 2009 end-page: 912 ident: CR34 article-title: Elovl4 5-bp deletion knock-in mouse model for Stargardt-like macular degeneration demonstrates accumulation of ELOVL4 and lipofuscin publication-title: Exp. Eye Res. doi: 10.1016/j.exer.2009.07.021 contributor: fullname: Vasireddy – volume: 23 start-page: 60 year: 2017 end-page: 89 ident: CR46 article-title: Appropriately differentiated ARPE-19 cells regain phenotype and gene expression profiles similar to those of native RPE cells publication-title: Mol. Vis. contributor: fullname: Samuel – volume: 11 start-page: 14 year: 2017 ident: CR44 article-title: Early Events in Retinal Degeneration Caused by Rhodopsin Mutation or Pigment Epithelium Malfunction: Differences and Similarities publication-title: Front. Neuroanat. doi: 10.3389/fnana.2017.00014 contributor: fullname: Di Pierdomenico – volume: 27 start-page: 983 year: 2006 end-page: 993 ident: CR42 article-title: Mitochondrial alterations of retinal pigment epithelium in age-related macular degeneration publication-title: Neurobiol. Aging doi: 10.1016/j.neurobiolaging.2005.05.012 contributor: fullname: Feher – volume: 46 start-page: 3031 year: 2005 end-page: 3038 ident: CR14 article-title: Correlation between inactive cathepsin D expression and retinal changes in mcd2/mcd2 transgenic mice publication-title: Invest. Ophthalmol. Vis. Sci. doi: 10.1167/iovs.04-1510 contributor: fullname: Zhang – volume: 126 start-page: 51 year: 2014 end-page: 60 ident: CR48 article-title: Understanding photoreceptor outer segment phagocytosis: use and utility of RPE cells in culture publication-title: Exp. Eye Res. doi: 10.1016/j.exer.2014.01.010 contributor: fullname: Finnemann – volume: 54 start-page: 1773 year: 2013 ident: CR20 article-title: Photoreceptor outer segment phagocytosis couples lysosome biogenesis to autophagy in the retinal pigment epithelium publication-title: Invest. Ophthalmol. Vis. Sci. contributor: fullname: Lakkaraju – volume: 292 start-page: 8038 year: 2017 end-page: 8047 ident: CR43 article-title: Phagocytosis-dependent ketogenesis in retinal pigment epithelium publication-title: J. Biol. Chem. doi: 10.1074/jbc.M116.770784 contributor: fullname: Reyes-Reveles – volume: 110 start-page: 5446 year: 2013 end-page: 5451 ident: CR5 article-title: Deciphering mutant ELOVL4 activity in autosomal-dominant Stargardt macular dystrophy publication-title: Proc. Natl. Acad. Sci. USA doi: 10.1073/pnas.1217251110 contributor: fullname: Logan – volume: 174 start-page: 2310 year: 2009 end-page: 2323 ident: CR37 article-title: Cooperative phagocytes: resident microglia and bone marrow immigrants remove dead photoreceptors in retinal lesions publication-title: Am. J. Pathol. doi: 10.2353/ajpath.2009.090023 contributor: fullname: Joly – volume: 11 start-page: 14 year: 2017 ident: 24357_CR44 publication-title: Front. Neuroanat. doi: 10.3389/fnana.2017.00014 contributor: fullname: J Di Pierdomenico – volume: 282 start-page: 117 year: 1998 ident: 24357_CR49 publication-title: Science doi: 10.1126/science.282.5386.117 contributor: fullname: SH Tsang – volume: 28 start-page: 348 year: 2009 ident: 24357_CR38 publication-title: Prog. Retin. Eye Res. doi: 10.1016/j.preteyeres.2009.06.001 contributor: fullname: H Xu – volume: 42 start-page: 1685 year: 2001 ident: 24357_CR32 publication-title: Invest. Ophthalmol. Vis. Sci. contributor: fullname: NL Mata – volume: 174 start-page: 2310 year: 2009 ident: 24357_CR37 publication-title: Am. J. Pathol. doi: 10.2353/ajpath.2009.090023 contributor: fullname: S Joly – volume: 42 start-page: 392 year: 1969 ident: 24357_CR11 publication-title: J. Cell Biol. doi: 10.1083/jcb.42.2.392 contributor: fullname: RW Young – volume: 53 start-page: 664 year: 2012 ident: 24357_CR4 publication-title: Invest. Ophthalmol. Vis. Sci. doi: 10.1167/iovs.11-8418 contributor: fullname: S Kuny – volume: 291 start-page: 6494 year: 2016 ident: 24357_CR31 publication-title: J. Biol. Chem. doi: 10.1074/jbc.M115.687004 contributor: fullname: S Sethna – volume: 16 start-page: 349 year: 2017 ident: 24357_CR35 publication-title: Aging Cell doi: 10.1111/acel.12561 contributor: fullname: P Shang – volume: 194 start-page: 1071 year: 1976 ident: 24357_CR45 publication-title: Science doi: 10.1126/science.982063 contributor: fullname: MM LaVail – volume: 11 start-page: 2301 year: 2016 ident: 24357_CR51 publication-title: Nat.Protoc. doi: 10.1038/nprot.2016.136 contributor: fullname: S Tyanova – volume: 56 start-page: 7109 year: 2015 ident: 24357_CR8 publication-title: Invest. Ophthalmol. Vis. Sci. doi: 10.1167/iovs.15-17567 contributor: fullname: S Kuny – volume: 325 start-page: 473 year: 2009 ident: 24357_CR21 publication-title: Science doi: 10.1126/science.1174447 contributor: fullname: M Sardiello – volume: 288 start-page: 15326 year: 2013 ident: 24357_CR40 publication-title: J. Biol. Chem. doi: 10.1074/jbc.M112.448712 contributor: fullname: H Kohno – volume: 110 start-page: 5181 year: 2013 ident: 24357_CR3 publication-title: Proc. Natl. Acad. Sci. USA doi: 10.1073/pnas.1214707110 contributor: fullname: P Barabas – volume: 58 start-page: 3826 year: 2017 ident: 24357_CR28 publication-title: Invest. Ophthalmol. Vis. Sci. doi: 10.1167/iovs.17-21929 contributor: fullname: WH Han – volume: 102 start-page: 4164 year: 2005 ident: 24357_CR29 publication-title: Proc. Natl. Acad. Sci. USA doi: 10.1073/pnas.0407698102 contributor: fullname: G Karan – volume: 54 start-page: 1773 year: 2013 ident: 24357_CR20 publication-title: Invest. Ophthalmol. Vis. Sci. contributor: fullname: J Xu – volume: 18 start-page: 562 year: 2004 ident: 24357_CR33 publication-title: FASEB J. doi: 10.1096/fj.03-0289fje contributor: fullname: M Bergmann – volume: 4 start-page: e7945 year: 2009 ident: 24357_CR39 publication-title: PLoS One. doi: 10.1371/journal.pone.0007945 contributor: fullname: W Ma – volume: 105 start-page: 12843 year: 2008 ident: 24357_CR1 publication-title: Proc. Natl. Acad. Sci. USA doi: 10.1073/pnas.0802607105 contributor: fullname: MP Agbaga – volume: 104 start-page: 4600 year: 2007 ident: 24357_CR10 publication-title: Proc. Natl. Acad. Sci. USA doi: 10.1073/pnas.0605818104 contributor: fullname: BL Krock – volume: 13 start-page: 566 year: 2007 ident: 24357_CR25 publication-title: Mol. Vis. contributor: fullname: J Yaung – volume: 23 start-page: 5 year: 2017 ident: 24357_CR41 publication-title: CNS Neurosci. Ther. doi: 10.1111/cns.12655 contributor: fullname: M Golpich – volume: 89 start-page: 905 year: 2009 ident: 24357_CR34 publication-title: Exp. Eye Res. doi: 10.1016/j.exer.2009.07.021 contributor: fullname: V Vasireddy – volume: 332 start-page: 1429 year: 2011 ident: 24357_CR23 publication-title: Science doi: 10.1126/science.1204592 contributor: fullname: C Settembre – volume: 92 start-page: 98 year: 2011 ident: 24357_CR36 publication-title: Exp. Eye Res. doi: 10.1016/j.exer.2010.11.008 contributor: fullname: PE Fort – volume: 81 start-page: 1345 year: 2007 ident: 24357_CR30 publication-title: J. Leukoc. Biol. doi: 10.1189/jlb.0207114 contributor: fullname: T Langmann – volume: 854 start-page: 717 year: 2016 ident: 24357_CR19 publication-title: Adv. Exp. Med. Biol. doi: 10.1007/978-3-319-17121-0_95 contributor: fullname: Y Mao – volume: 23 start-page: 60 year: 2017 ident: 24357_CR46 publication-title: Mol. Vis. contributor: fullname: W Samuel – volume: 51 start-page: 2248 year: 2010 ident: 24357_CR18 publication-title: Invest. Ophthalmol. Vis. Sci. doi: 10.1167/iovs.09-4718 contributor: fullname: S Kuny – volume: 2 start-page: e106 year: 2014 ident: 24357_CR17 publication-title: Lancet Glob. Health doi: 10.1016/S2214-109X(13)70145-1 contributor: fullname: WL Wong – volume: 102 start-page: 11846 year: 2005 ident: 24357_CR24 publication-title: Proc. Natl. Acad. Sci. USA doi: 10.1073/pnas.0504759102 contributor: fullname: J Tian – volume: 46 start-page: 3031 year: 2005 ident: 24357_CR14 publication-title: Invest. Ophthalmol. Vis. Sci. doi: 10.1167/iovs.04-1510 contributor: fullname: D Zhang – volume: 110 start-page: 5446 year: 2013 ident: 24357_CR5 publication-title: Proc. Natl. Acad. Sci. USA doi: 10.1073/pnas.1217251110 contributor: fullname: S Logan – volume: 90 start-page: 299 year: 2012 ident: 24357_CR16 publication-title: Acta Ophthalmol. doi: 10.1111/j.1755-3768.2011.02179.x contributor: fullname: K Kinnunen – volume: 31 start-page: 121 year: 2012 ident: 24357_CR9 publication-title: Prog. Retin. Eye Res. doi: 10.1016/j.preteyeres.2011.12.001 contributor: fullname: JR Sparrow – volume: 201 start-page: 1129 year: 1998 ident: 24357_CR27 publication-title: J. Exp. Biol. doi: 10.1242/jeb.201.8.1129 contributor: fullname: E Gnaiger – volume: 31 start-page: 1095 year: 2012 ident: 24357_CR22 publication-title: EMBO J. doi: 10.1038/emboj.2012.32 contributor: fullname: C Settembre – volume: 27 start-page: 983 year: 2006 ident: 24357_CR42 publication-title: Neurobiol. Aging doi: 10.1016/j.neurobiolaging.2005.05.012 contributor: fullname: J Feher – volume: 126 start-page: 51 year: 2014 ident: 24357_CR48 publication-title: Exp. Eye Res. doi: 10.1016/j.exer.2014.01.010 contributor: fullname: F Mazzoni – volume: 55 start-page: 3669 year: 2014 ident: 24357_CR7 publication-title: Invest. Ophthalmol. Vis. Sci. doi: 10.1167/iovs.13-13099 contributor: fullname: MP Agbaga – volume: 292 start-page: 8038 year: 2017 ident: 24357_CR43 publication-title: J. Biol. Chem. doi: 10.1074/jbc.M116.770784 contributor: fullname: J Reyes-Reveles – volume: 2 start-page: 981 year: 2010 ident: 24357_CR13 publication-title: Aging (Albany N.Y.) contributor: fullname: E Picard – volume: 99 start-page: 14682 year: 2002 ident: 24357_CR26 publication-title: Proc. Natl. Acad. Sci. USA doi: 10.1073/pnas.222551899 contributor: fullname: JW Crabb – volume: 280 start-page: 32521 year: 2005 ident: 24357_CR2 publication-title: J. Biol. Chem. doi: 10.1074/jbc.M503411200 contributor: fullname: C Grayson – volume: 935 start-page: 285 year: 2013 ident: 24357_CR47 publication-title: Methods Mol. Biol. doi: 10.1007/978-1-62703-080-9_20 contributor: fullname: Y Mao – volume: 145 start-page: 450 year: 2016 ident: 24357_CR50 publication-title: Exp. Eye Res. doi: 10.1016/j.exer.2015.09.015 contributor: fullname: H Wei – volume: 124 start-page: 523 year: 2011 ident: 24357_CR15 publication-title: J. Cell Sci. doi: 10.1242/jcs.078790 contributor: fullname: JS Zigler – volume: 44 start-page: 826 year: 2003 ident: 24357_CR12 publication-title: Invest. Ophthalmol. Vis. Sci. doi: 10.1167/iovs.02-0438 contributor: fullname: JL Duncan – volume: 55 start-page: 3150 year: 2014 ident: 24357_CR6 publication-title: Invest. Ophthalmol. Vis. Sci. doi: 10.1167/iovs.14-13995 contributor: fullname: LD Bennett |
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Snippet | For many neurodegenerative disorders, expression of a pathological protein by one cell type impedes function of other cell types, which in turn contributes to... Abstract For many neurodegenerative disorders, expression of a pathological protein by one cell type impedes function of other cell types, which in turn... Abstract For many neurodegenerative disorders, expression of a pathological protein by one cell type impedes function of other cell types, which in turn... |
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SubjectTerms | 13/1 13/106 13/51 14/19 38/77 38/79 631/378/2613/1786 631/80/474/1624 64/60 82/58 Cathepsin D Cell death Cytotoxicity Epithelium Humanities and Social Sciences Macrophages Microglia multidisciplinary Neurodegenerative diseases Oxidative stress Phagocytes Phagosomes Photoreceptors Retina Retinal pigment epithelium Rodents Science Science (multidisciplinary) Transgenic mice |
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Title | Photoreceptor-induced RPE phagolysosomal maturation defects in Stargardt-like Maculopathy (STGD3) |
URI | https://link.springer.com/article/10.1038/s41598-018-24357-4 https://www.ncbi.nlm.nih.gov/pubmed/29654292 https://www.proquest.com/docview/2024479407 https://search.proquest.com/docview/2025318464 https://pubmed.ncbi.nlm.nih.gov/PMC5899129 https://doaj.org/article/a678339708ca45858f64f495a3c5912a |
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