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Natl. Acad. Sci. U.S.A."],"published-print":{"date-parts":[[2018,11,20]]},"abstract":"<jats:title>Significance<\/jats:title>\n                  <jats:p>\n                    Recessive Stargardt macular degeneration (STGD1) and a subset of cone\u2013rod dystrophies are caused by mutations in the\n                    <jats:italic>Abca4<\/jats:italic>\n                    gene. The ABCA4 protein is a flippase in photoreceptor cells that helps eliminate retinaldehyde, a toxic photoproduct of vision. Here we found that ABCA4 is additionally present in the retinal pigment epithelium (RPE) of mice at approximately 1% of its abundance in the neural retina. Genetically modified mice that express ABCA4 in RPE but not in photoreceptor cells showed partial rescue of both the lipofuscin accumulation and photoreceptor degeneration observed in\n                    <jats:italic>Abca4<\/jats:italic>\n                    <jats:sup>\n                      <jats:italic>\u2212\/\u2212<\/jats:italic>\n                    <\/jats:sup>\n                    mice and in STGD1 patients. These observations suggest that ABCA4 in the RPE prevents photoreceptor degeneration in\n                    <jats:italic>Abca4<\/jats:italic>\n                    <jats:sup>\n                      <jats:italic>\u2212\/\u2212<\/jats:italic>\n                    <\/jats:sup>\n                    mice and possibly in STGD1 patients.\n                  <\/jats:p>","DOI":"10.1073\/pnas.1802519115","type":"journal-article","created":{"date-parts":[[2018,11,5]],"date-time":"2018-11-05T15:14:23Z","timestamp":1541430863000},"update-policy":"https:\/\/doi.org\/10.1073\/pnas.cm10313","source":"Crossref","is-referenced-by-count":154,"title":["Expression of ABCA4 in the retinal pigment epithelium and its implications for Stargardt macular degeneration"],"prefix":"10.1073","volume":"115","author":[{"given":"Tamara L.","family":"Lenis","sequence":"first","affiliation":[{"name":"Stein Eye Institute, Department of Ophthalmology, David Geffen School of Medicine, University of California, Los Angeles, CA 90095;"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jane","family":"Hu","sequence":"additional","affiliation":[{"name":"Stein Eye Institute, Department of Ophthalmology, David Geffen School of Medicine, University of California, Los Angeles, CA 90095;"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sze Yin","family":"Ng","sequence":"additional","affiliation":[{"name":"Stein Eye Institute, Department of Ophthalmology, David Geffen School of Medicine, University of California, Los Angeles, CA 90095;"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Zhichun","family":"Jiang","sequence":"additional","affiliation":[{"name":"Stein Eye Institute, Department of Ophthalmology, David Geffen School of Medicine, University of California, Los Angeles, CA 90095;"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Shanta","family":"Sarfare","sequence":"additional","affiliation":[{"name":"Stein Eye Institute, Department of Ophthalmology, David Geffen School of Medicine, University of California, Los Angeles, CA 90095;"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marcia B.","family":"Lloyd","sequence":"additional","affiliation":[{"name":"Stein Eye Institute, Department of Ophthalmology, David Geffen School of Medicine, University of California, Los Angeles, CA 90095;"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Nicholas J.","family":"Esposito","sequence":"additional","affiliation":[{"name":"Department of Biological Sciences, Fordham University, Bronx, NY 10458;"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"William","family":"Samuel","sequence":"additional","affiliation":[{"name":"Laboratory of Retinal Cell and Molecular Biology, National Eye Institute, National Institutes of Health, Bethesda, MD 20814;"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Cynthia","family":"Jaworski","sequence":"additional","affiliation":[{"name":"Laboratory of Retinal Cell and Molecular Biology, National Eye Institute, National Institutes of Health, Bethesda, MD 20814;"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Dean","family":"Bok","sequence":"additional","affiliation":[{"name":"Stein Eye Institute, Department of Ophthalmology, David Geffen School of Medicine, University of California, Los Angeles, CA 90095;"},{"name":"Department of Neurobiology, David Geffen School of Medicine, University of California, Los Angeles, CA 90095;"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-9298-0736","authenticated-orcid":false,"given":"Silvia C.","family":"Finnemann","sequence":"additional","affiliation":[{"name":"Department of Biological Sciences, Fordham University, Bronx, NY 10458;"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Monte J.","family":"Radeke","sequence":"additional","affiliation":[{"name":"Neuroscience Research Institute, University of California, Santa Barbara, CA 93106;"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-1813-5291","authenticated-orcid":false,"given":"T. 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