{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,13]],"date-time":"2026-10-13T22:00:37Z","timestamp":1791928837135,"version":"4.3.4"},"reference-count":67,"publisher":"Association for Research in Vision and Ophthalmology (ARVO)","issue":"13","license":[{"start":{"date-parts":[[2024,11,13]],"date-time":"2024-11-13T00:00:00Z","timestamp":1731456000000},"content-version":"unspecified","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Invest. Ophthalmol. Vis. Sci."],"published-print":{"date-parts":[[2024,11,13]]},"DOI":"10.1167\/iovs.65.13.23","type":"journal-article","created":{"date-parts":[[2024,11,13]],"date-time":"2024-11-13T11:41:58Z","timestamp":1731498118000},"page":"23","source":"Crossref","is-referenced-by-count":7,"title":["Genetic and Cellular Basis of Impaired Phagocytosis and Photoreceptor Degeneration in CLN3 Disease"],"prefix":"10.1167","volume":"65","author":[{"given":"Jimin","family":"Han","sequence":"first","affiliation":[{"name":"Department of Ophthalmology, University of Rochester, Rochester, New York, United States"},{"name":"Department of Biomedical Genetics, University of Rochester, Rochester, New York, United States"},{"name":"Center for Visual Science, University of Rochester, Rochester, New York, United States"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sueanne","family":"Chear","sequence":"additional","affiliation":[{"name":"Wicking Dementia Research and Education Centre, University of Tasmania, Tasmania, Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jana","family":"Talbot","sequence":"additional","affiliation":[{"name":"Wicking Dementia Research and Education Centre, University of Tasmania, Tasmania, Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Vicki","family":"Swier","sequence":"additional","affiliation":[{"name":"Pediatrics & Rare Diseases Group; Sanford Research, Sioux Falls, South Dakota, United States"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Clarissa","family":"Booth","sequence":"additional","affiliation":[{"name":"Pediatrics & Rare Diseases Group; Sanford Research, Sioux Falls, South Dakota, United States"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Cheyenne","family":"Reuben-Thomas","sequence":"additional","affiliation":[{"name":"Department of Ophthalmology, University of Rochester, Rochester, New York, United States"},{"name":"Department of Biomedical Genetics, University of Rochester, Rochester, New York, United States"},{"name":"Center for Visual Science, University of Rochester, Rochester, New York, United States"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sonal","family":"Dalvi","sequence":"additional","affiliation":[{"name":"Department of Ophthalmology, University of Rochester, Rochester, New York, United States"},{"name":"Department of Biomedical Genetics, University of Rochester, Rochester, New York, United States"},{"name":"Center for Visual Science, University of Rochester, Rochester, New York, United States"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jill M.","family":"Weimer","sequence":"additional","affiliation":[{"name":"Pediatrics & Rare Diseases Group; Sanford Research, Sioux Falls, South Dakota, United States"},{"name":"Department of Pediatrics; Sanford School of Medicine, University of South Dakota, Sioux Falls, South Dakota, United States"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Alex W.","family":"Hewitt","sequence":"additional","affiliation":[{"name":"Menzies Institute for Medical Research, University of Tasmania, Tasmania, Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Anthony L.","family":"Cook","sequence":"additional","affiliation":[{"name":"Wicking Dementia Research and Education Centre, University of Tasmania, Tasmania, Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ruchira","family":"Singh","sequence":"additional","affiliation":[{"name":"Department of Ophthalmology, University of Rochester, Rochester, New York, United States"},{"name":"Department of Biomedical Genetics, University of Rochester, Rochester, New York, United States"},{"name":"Center for Visual Science, University of Rochester, Rochester, New York, United States"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"27","reference":[{"issue":"11","key":"bib1","doi-asserted-by":"publisher","first-page":"1795","DOI":"10.1016\/j.bbadis.2012.08.012","article-title":"The neuronal ceroid-lipofuscinoses: a historical introduction","volume":"1832","author":"Haltia","year":"2013","journal-title":"Biochim Biophys Acta"},{"issue":"11","key":"bib2","doi-asserted-by":"publisher","first-page":"1807","DOI":"10.1016\/j.bbadis.2012.11.014","article-title":"Human pathology in NCL","volume":"1832","author":"Anderson","year":"2013","journal-title":"Biochim Biophys Acta"},{"issue":"3","key":"bib3","doi-asserted-by":"publisher","first-page":"107","DOI":"10.1007\/s10048-005-0218-3","article-title":"Correlations between genotype, ultrastructural morphology and clinical phenotype in the neuronal ceroid lipofuscinoses","volume":"6","author":"Mole","year":"2005","journal-title":"Neurogenetics"},{"issue":"11\u201312","key":"bib4","doi-asserted-by":"crossref","first-page":"1253","DOI":"10.2119\/molmed.2010.00241","article-title":"Analysis of potential biomarkers and modifier genes affecting the clinical course of CLN3 disease","volume":"17","author":"Lebrun","year":"2011","journal-title":"Mol Med"},{"issue":"3","key":"bib5","doi-asserted-by":"publisher","first-page":"241","DOI":"10.1007\/s10633-023-09930-1","article-title":"Early recognition of CLN3 disease facilitated by visual electrophysiology and multimodal imaging","volume":"146","author":"Sakti","year":"2023","journal-title":"Doc Ophthalmol"},{"issue":"11","key":"bib6","doi-asserted-by":"publisher","first-page":"1801","DOI":"10.1016\/j.bbadis.2013.04.008","article-title":"NCL diseases - clinical perspectives","volume":"1832","author":"Schulz","year":"2013","journal-title":"Biochim Biophys Acta"},{"issue":"5","key":"bib7","doi-asserted-by":"publisher","first-page":"321","DOI":"10.1006\/nbdi.1999.0267","article-title":"Targeted disruption of the Cln3 gene provides a mouse model for Batten disease","volume":"6","author":"Mitchison","year":"1999","journal-title":"Neurobiol Dis"},{"issue":"6","key":"bib8","doi-asserted-by":"publisher","first-page":"648","DOI":"10.1016\/j.neuint.2011.02.003","article-title":"Altered sensitivity of cerebellar granule cells to glutamate receptor overactivation in the Cln3(?ex7\/8)-knock-in mouse model of juvenile neuronal ceroid lipofuscinosis","volume":"58","author":"Finn","year":"2011","journal-title":"Neurochem Int"},{"issue":"22","key":"bib9","doi-asserted-by":"publisher","first-page":"2709","DOI":"10.1093\/hmg\/11.22.2709","article-title":"Cln3(Deltaex7\/8) knock-in mice with the common JNCL mutation exhibit progressive neurologic disease that begins before birth","volume":"11","author":"Cotman","year":"2002","journal-title":"Hum Mol Genet"},{"key":"bib10","doi-asserted-by":"publisher","first-page":"93","DOI":"10.1016\/j.brainres.2009.02.009","article-title":"Cerebellar defects in a mouse model of juvenile neuronal ceroid lipofuscinosis","volume":"1266","author":"Weimer","year":"2009","journal-title":"Brain Res"},{"issue":"2","key":"bib11","doi-asserted-by":"publisher","first-page":"231","DOI":"10.1016\/j.brainres.2004.07.030","article-title":"Late onset neurodegeneration in the Cln3-\/- mouse model of juvenile neuronal ceroid lipofuscinosis is preceded by low level glial activation","volume":"1023","author":"Pontikis","year":"2004","journal-title":"Brain Res"},{"issue":"10","key":"bib12","doi-asserted-by":"publisher","first-page":"165883","DOI":"10.1016\/j.bbadis.2020.165883","article-title":"Loss of CLN3, the gene mutated in juvenile neuronal ceroid lipofuscinosis, leads to metabolic impairment and autophagy induction in retinal pigment epithelium","volume":"1866","author":"Zhong","year":"2020","journal-title":"Biochim Biophys Acta Mol Basis Dis"},{"issue":"24","key":"bib13","first-page":"7060","article-title":"Photoreceptor phagosome processing defects and disturbed autophagy in retinal pigment epithelium of Cln3?ex1-6 mice modelling juvenile neuronal ceroid lipofuscinosis (Batten disease)","volume":"24","author":"Wavre-Shapton","year":"2015","journal-title":"Hum Mol Genet"},{"issue":"4","key":"bib14","doi-asserted-by":"publisher","first-page":"515","DOI":"10.1006\/mcne.2001.1099","article-title":"Retinal pathology and function in a Cln3 knockout mouse model of juvenile neuronal ceroid lipofuscinosis (Batten disease)","volume":"19","author":"Seigel","year":"2002","journal-title":"Mol Cell Neurosci"},{"issue":"8","key":"bib15","doi-asserted-by":"publisher","first-page":"dmm050038","DOI":"10.1242\/dmm.050038","article-title":"A novel porcine model of CLN3 Batten disease recapitulates clinical phenotypes","volume":"16","author":"Swier","year":"2023","journal-title":"Dis Model Mech"},{"issue":"4","key":"bib16","doi-asserted-by":"publisher","first-page":"576","DOI":"10.1016\/j.neurobiolaging.2005.12.006","article-title":"You say lipofuscin, we say ceroid: defining autofluorescent storage material","volume":"27","author":"Seehafer","year":"2006","journal-title":"Neurobiol Aging"},{"issue":"7","key":"bib17","doi-asserted-by":"publisher","first-page":"499","DOI":"10.1016\/j.braindev.2008.12.008","article-title":"Towards understanding the neuronal ceroid lipofuscinoses","volume":"31","author":"Kohlsch\u00fctter","year":"2009","journal-title":"Brain Dev"},{"issue":"9","key":"bib18","doi-asserted-by":"publisher","first-page":"1746","DOI":"10.1016\/S0161-6420(00)00264-5","article-title":"Histopathologic and immunocytochemical analysis of the retina and ocular tissues in Batten disease","volume":"107","author":"Bensaoula","year":"2000","journal-title":"Ophthalmology"},{"issue":"2","key":"bib20","doi-asserted-by":"publisher","first-page":"242","DOI":"10.1016\/j.nbd.2007.08.017","article-title":"Phenotypic characterization of a mouse model of juvenile neuronal ceroid lipofuscinosis","volume":"29","author":"Katz","year":"2008","journal-title":"Neurobiol Dis"},{"issue":"1","key":"bib21","doi-asserted-by":"publisher","first-page":"55","DOI":"10.1111\/nyas.12990","article-title":"Vision loss in juvenile neuronal ceroid lipofuscinosis (CLN3 disease)","volume":"1371","author":"Ouseph","year":"2016","journal-title":"Ann N Y Acad Sci"},{"issue":"1","key":"bib22","doi-asserted-by":"publisher","first-page":"161","DOI":"10.1038\/s42003-021-01682-5","article-title":"A human model of Batten disease shows role of CLN3 in phagocytosis at the photoreceptor-RPE interface","volume":"4","author":"Tang","year":"2021","journal-title":"Commun Biol"},{"issue":"4","key":"bib23","doi-asserted-by":"publisher","first-page":"463","DOI":"10.1016\/j.survophthal.2009.04.007","article-title":"Juvenile neuronal ceroid lipofuscinosis (JNCL) and the eye","volume":"54","author":"Bozorg","year":"2009","journal-title":"Surv Ophthalmol"},{"issue":"4","key":"bib24","doi-asserted-by":"publisher","first-page":"576","DOI":"10.1016\/j.neurobiolaging.2005.12.006","article-title":"You say lipofuscin, we say ceroid: defining autofluorescent storage material","volume":"27","author":"Seehafer","year":"2006","journal-title":"Neurobiology of Aging"},{"issue":"7\u20138","key":"bib25","doi-asserted-by":"crossref","first-page":"348","DOI":"10.1007\/s00335-017-9684-9","article-title":"Editing the genome of hiPSC with CRISPR\/Cas9: disease models","volume":"28","author":"Bassett","year":"2017","journal-title":"Mamm Genome"},{"issue":"R2","key":"bib26","doi-asserted-by":"publisher","first-page":"R187","DOI":"10.1093\/hmg\/ddab219","article-title":"Linking genome variants to disease: scalable approaches to test the functional impact of human mutations","volume":"30","author":"Findlay","year":"2021","journal-title":"Hum Mol Genet"},{"issue":"4","key":"bib27","doi-asserted-by":"publisher","first-page":"861","DOI":"10.1038\/s12276-024-01212-3","article-title":"Recent advances in CRISPR-based functional genomics for the study of disease-associated genetic variants","volume":"56","author":"Kim","year":"2024","journal-title":"Exp Mol Med"},{"key":"bib28","doi-asserted-by":"publisher","first-page":"101248","DOI":"10.1016\/j.preteyeres.2024.101248","article-title":"Pluripotent stem cell-derived models of retinal disease: elucidating pathogenesis, evaluating novel treatments, and estimating toxicity","volume":"100","author":"Kurzawa-Akanbi","year":"2024","journal-title":"Prog Retin Eye Res"},{"key":"bib29","doi-asserted-by":"publisher","first-page":"522","DOI":"10.1016\/j.omtm.2023.05.012","article-title":"CRISPR-Cas9 correction of a nonsense mutation in","volume":"29","author":"Afanasyeva","year":"2023","journal-title":"Mol Ther Methods Clin Dev"},{"issue":"4","key":"bib30","doi-asserted-by":"publisher","first-page":"101459","DOI":"10.1016\/j.xcrm.2024.101459","article-title":"CRISPR editing of anti-anemia drug target rescues independent preclinical models of retinitis pigmentosa","volume":"5","author":"Nolan","year":"2024","journal-title":"Cell Rep Med"},{"issue":"4","key":"bib31","doi-asserted-by":"publisher","first-page":"43","DOI":"10.1167\/iovs.65.4.43","article-title":"CFH Haploinsufficiency and complement alterations in early-onset macular degeneration","volume":"65","author":"Lim","year":"2024","journal-title":"Invest Ophthalmol Vis Sci"},{"issue":"39","key":"bib32","doi-asserted-by":"publisher","first-page":"E8214","DOI":"10.1073\/pnas.1710430114","article-title":"Drusen in patient-derived hiPSC-RPE models of macular dystrophies","volume":"114","author":"Galloway","year":"2017","journal-title":"Proc Natl Acad Sci USA"},{"issue":"4","key":"bib33","doi-asserted-by":"publisher","first-page":"577","DOI":"10.1007\/s00439-023-02531-7","article-title":"Biallelic CLCN2 mutations cause retinal degeneration by impairing retinal pigment epithelium phagocytosis and chloride channel function","volume":"142","author":"Xu","year":"2023","journal-title":"Hum Genet"},{"issue":"4","key":"bib34","doi-asserted-by":"publisher","first-page":"414","DOI":"10.1002\/stem.3325","article-title":"NRL","volume":"39","author":"Cuevas","year":"2021","journal-title":"Stem Cells"},{"key":"bib35","doi-asserted-by":"publisher","first-page":"18","DOI":"10.1016\/j.ymeth.2021.02.009","article-title":"Use of CRISPR\/Cas ribonucleoproteins for high throughput gene editing of induced pluripotent stem cells","volume":"194","author":"Wang","year":"2021","journal-title":"Methods"},{"issue":"12","key":"bib36","doi-asserted-by":"publisher","first-page":"dmm049651","DOI":"10.1242\/dmm.049651","article-title":"Lysosomal alterations and decreased electrophysiological activity in CLN3 disease patient-derived cortical neurons","volume":"15","author":"Chear","year":"2022","journal-title":"Dis Model Mech"},{"issue":"11","key":"bib37","doi-asserted-by":"publisher","first-page":"1665","DOI":"10.1101\/gr.6861907","article-title":"PennCNV: an integrated hidden Markov model designed for high-resolution copy number variation detection in whole-genome SNP genotyping data","volume":"17","author":"Wang","year":"2007","journal-title":"Genome Res"},{"issue":"6","key":"bib38","doi-asserted-by":"publisher","first-page":"2013","DOI":"10.1093\/nar\/gkm076","article-title":"QuantiSNP: an objective Bayes hidden-Markov model to detect and accurately map copy number variation using SNP genotyping data","volume":"35","author":"Colella","year":"2007","journal-title":"Nucleic Acids Res"},{"issue":"1","key":"bib39","doi-asserted-by":"publisher","first-page":"76","DOI":"10.1186\/s13059-021-02293-3","article-title":"Single cell eQTL analysis identifies cell type-specific genetic control of gene expression in fibroblasts and reprogrammed induced pluripotent stem cells","volume":"22","author":"Neavin","year":"2021","journal-title":"Genome Biol"},{"issue":"5","key":"bib40","doi-asserted-by":"publisher","first-page":"846","DOI":"10.1016\/j.stem.2021.02.006","article-title":"3D iPSC modeling of the retinal pigment epithelium-choriocapillaris complex identifies factors involved in the pathology of macular degeneration","volume":"28","author":"Manian","year":"2021","journal-title":"Cell Stem Cell"},{"key":"bib41","doi-asserted-by":"publisher","first-page":"74","DOI":"10.1016\/j.exer.2014.05.003","article-title":"A detailed three-step protocol for live imaging of intracellular traffic in polarized primary porcine RPE monolayers","volume":"124","author":"Toops","year":"2014","journal-title":"Exp Eye Res"},{"issue":"94","key":"bib42","first-page":"e52100","article-title":"Large-scale purification of porcine or bovine photoreceptor outer segments for phagocytosis assays on retinal pigment epithelial cells","author":"Parinot","year":"2014","journal-title":"J Vis Exp"},{"issue":"3","key":"bib43","doi-asserted-by":"publisher","first-page":"593","DOI":"10.1093\/hmg\/dds469","article-title":"iPS cell modeling of Best disease: insights into the pathophysiology of an inherited macular degeneration","volume":"22","author":"Singh","year":"2013","journal-title":"Hum Mol Genet"},{"issue":"3","key":"bib44","doi-asserted-by":"publisher","first-page":"1075","DOI":"10.1016\/j.stemcr.2018.01.017","article-title":"Quantified F-actin morphology is predictive of phagocytic capacity of stem cell-derived retinal pigment epithelium","volume":"10","author":"M\u00fcller","year":"2018","journal-title":"Stem Cell Rep"},{"key":"bib45","doi-asserted-by":"publisher","first-page":"101918","DOI":"10.1016\/j.redox.2021.101918","article-title":"Lack of the antioxidant enzyme methionine sulfoxide reductase A in mice impairs RPE phagocytosis and causes photoreceptor cone dysfunction","volume":"42","author":"Mazzoni","year":"2021","journal-title":"Redox Biol"},{"issue":"7","key":"bib46","doi-asserted-by":"publisher","first-page":"3661","DOI":"10.3390\/ijms23073661","article-title":"Probing photoreceptor outer segment phagocytosis by the RPE in vivo: models and methodologies","volume":"23","author":"Vargas","year":"2022","journal-title":"Int J Mol Sci"},{"key":"bib47","doi-asserted-by":"publisher","first-page":"245","DOI":"10.1007\/978-1-62703-080-9","article-title":"Analysis of photoreceptor rod outer segment phagocytosis by RPE cells in situ","volume":"935","author":"Sethna","year":"2013","journal-title":"Methods Mol Biol"},{"key":"bib48","doi-asserted-by":"publisher","first-page":"96","DOI":"10.1038\/s41420-019-0171-9","article-title":"Environmental stress impairs photoreceptor outer segment (POS) phagocytosis and degradation and induces autofluorescent material accumulation in hiPSC-RPE cells","volume":"5","author":"Dalvi","year":"2019","journal-title":"Cell Death Discov"},{"issue":"38","key":"bib49","doi-asserted-by":"publisher","first-page":"22378","DOI":"10.1074\/jbc.270.38.22378","article-title":"A natural disruption of the secretory group II phospholipase A2 gene in inbred mouse strains","volume":"270","author":"Kennedy","year":"1995","journal-title":"J Biol Chem"},{"issue":"2","key":"bib50","doi-asserted-by":"publisher","first-page":"271","DOI":"10.1006\/dbio.2000.9912","article-title":"Clonally derived human embryonic stem cell lines maintain pluripotency and proliferative potential for prolonged periods of culture","volume":"227","author":"Amit","year":"2000","journal-title":"Dev Biol"},{"issue":"7","key":"bib51","doi-asserted-by":"publisher","first-page":"2792","DOI":"10.1167\/iovs.17-23157","article-title":"Characterization of human iPSC-RPE on a prosthetic Bruch's membrane manufactured from silk fibroin","volume":"59","author":"Galloway","year":"2018","journal-title":"Invest Ophthalmol Vis Sci"},{"issue":"5","key":"bib52","first-page":"863","article-title":"Isolation and provisional identification of plasma membrane populations from cultured human retinal pigment epithelium","volume":"31","author":"Mircheff","year":"1990","journal-title":"Invest Ophthalmol Vis Sci"},{"issue":"5","key":"bib53","doi-asserted-by":"publisher","first-page":"662","DOI":"10.1038\/nprot.2009.33","article-title":"A protocol for the culture and differentiation of highly polarized human retinal pigment epithelial cells","volume":"4","author":"Sonoda","year":"2009","journal-title":"Nat Protoc"},{"issue":"10, Part B","key":"bib54","doi-asserted-by":"publisher","first-page":"2262","DOI":"10.1016\/j.bbadis.2015.05.007","article-title":"Human NCL neuropathology","volume":"1852","author":"Radke","year":"2015","journal-title":"Biochim Biophys Acta"},{"issue":"2","key":"bib55","doi-asserted-by":"publisher","first-page":"e55526","DOI":"10.1371\/journal.pone.0055526","article-title":"Alterations in ROS activity and lysosomal pH account for distinct patterns of macroautophagy in LINCL and JNCL fibroblasts","volume":"8","author":"Vidal-Donet","year":"2013","journal-title":"PLoS One"},{"issue":"13","key":"bib56","doi-asserted-by":"publisher","first-page":"2882","DOI":"10.1002\/jnr.21416","article-title":"Progressive oxidative damage in the central nervous system of a murine model for juvenile Batten disease","volume":"85","author":"Benedict","year":"2007","journal-title":"J Neurosci Res"},{"issue":"10","key":"bib57","doi-asserted-by":"publisher","first-page":"2037","DOI":"10.1093\/hmg\/ddr088","article-title":"The Batten disease gene CLN3 is required for the response to oxidative stress","volume":"20","author":"Tuxworth","year":"2011","journal-title":"Hum Mol Genet"},{"issue":"9","key":"bib58","doi-asserted-by":"publisher","first-page":"dmm033597","DOI":"10.1242\/dmm.033597","article-title":"Immunomodulation with minocycline rescues retinal degeneration in juvenile neuronal ceroid lipofuscinosis mice highly susceptible to light damage","volume":"11","author":"Dannhausen","year":"2018","journal-title":"Dis Model Mech"},{"issue":"8","key":"bib59","doi-asserted-by":"publisher","first-page":"1889","DOI":"10.1016\/j.ymthe.2017.04.021","article-title":"Fingolimod and teriflunomide attenuate neurodegeneration in mouse models of neuronal ceroid lipofuscinosis","volume":"25","author":"Groh","year":"2017","journal-title":"Mol Ther"},{"issue":"1","key":"bib60","doi-asserted-by":"publisher","first-page":"17","DOI":"10.1016\/S1096-7192(02)00201-9","article-title":"Functional categorization of gene expression changes in the cerebellum of a Cln3-knockout mouse model for Batten disease","volume":"78","author":"Brooks","year":"2003","journal-title":"Mol Genet Metab"},{"issue":"13","key":"bib61","first-page":"494","article-title":"Kinetics of oxidized rod outer segment phagocytosis under chronic oxidative stress in an in-vitro model of age-related macular degeneration","volume":"51","author":"Gupta","year":"2010","journal-title":"Invest Ophthalmol Vis Sci"},{"issue":"2","key":"bib62","doi-asserted-by":"publisher","first-page":"374","DOI":"10.1006\/excr.1994.1157","article-title":"Superoxide inhibits proliferation and phagocytic internalization of photoreceptor outer segments by bovine retinal pigment epithelium in vitro","volume":"212","author":"Becquet","year":"1994","journal-title":"Exp Cell Res"},{"key":"bib63","doi-asserted-by":"publisher","first-page":"179","DOI":"10.3389\/fncel.2020.00179","article-title":"Limitations and promise of retinal tissue from human pluripotent stem cells for developing therapies of blindness","volume":"14","author":"Singh","year":"2020","journal-title":"Front Cell Neurosci"},{"key":"bib64","doi-asserted-by":"crossref","first-page":"dev171686","DOI":"10.1242\/dev.171686","article-title":"Reproducibility and staging of 3D human retinal organoids across multiple pluripotent stem cell lines","volume":"146","author":"Capowski","year":"2018","journal-title":"Development"},{"issue":"10 Pt B","key":"bib65","doi-asserted-by":"crossref","first-page":"2262","DOI":"10.1016\/j.bbadis.2015.05.007","article-title":"Human NCL neuropathology","volume":"1852","author":"Radke","year":"2015","journal-title":"Biochim Biophys Acta"},{"issue":"8","key":"bib66","doi-asserted-by":"publisher","first-page":"642","DOI":"10.1002\/term.1458","article-title":"Differentiation of human pluripotent stem cells to retinal pigmented epithelium in defined conditions using purified extracellular matrix proteins","volume":"7","author":"Rowland","year":"2013","journal-title":"J Tissue Eng Regen Med"},{"key":"bib67","doi-asserted-by":"publisher","first-page":"1","DOI":"10.1007\/978-3-030-28471-8","article-title":"Pluripotent stem cells to model degenerative retinal diseases: the RPE perspective","volume":"1186","author":"Dalvi","year":"2019","journal-title":"Adv Exp Med Biol"},{"key":"bib19","article-title":"Neuronal Ceroid-Lipofuscinoses \u2013 RETIRED CHAPTER, FOR HISTORICAL REFERENCE ONLY. 2001 Oct 10 [Updated 2013 Aug 1].","volume-title":"GeneReviews [Internet].","author":"Mole","year":"1993\u20132024"}],"container-title":["Investigative Ophthalmology &amp; Visual Science"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/iovs.arvojournals.org\/article.aspx?articleid=2802225","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,10,13]],"date-time":"2026-10-13T21:31:50Z","timestamp":1791927110000},"score":1,"resource":{"primary":{"URL":"https:\/\/iovs.arvojournals.org\/article.aspx?articleid=2802225"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2024,11,13]]},"references-count":67,"journal-issue":{"issue":"13","published-print":{"date-parts":[[2024,11,4]]}},"URL":"https:\/\/doi.org\/10.1167\/iovs.65.13.23","relation":{"has-preprint":[{"id-type":"doi","id":"10.1101\/2024.06.09.597388","asserted-by":"object"}]},"ISSN":["1552-5783"],"issn-type":[{"value":"1552-5783","type":"print"}],"subject":[],"published":{"date-parts":[[2024,11,13]]}}}