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The <jats:styled-content style=\"fixed-case\">RPE<\/jats:styled-content> adopts either a smooth or mottled appearance on fundus autofluorescence (<jats:styled-content style=\"fixed-case\">FAF<\/jats:styled-content>). It is not known how <jats:styled-content style=\"fixed-case\">RPE<\/jats:styled-content> changes predict the health of the overlying ellipsoid zone (<jats:styled-content style=\"fixed-case\">EZ<\/jats:styled-content>).<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>A retrospective review of <jats:styled-content style=\"fixed-case\">FAF<\/jats:styled-content> and optical coherence tomography (<jats:styled-content style=\"fixed-case\">OCT<\/jats:styled-content>) images from 20 patients with choroideremia was performed. The percentage of intact <jats:styled-content style=\"fixed-case\">EZ<\/jats:styled-content> in each smooth and mottled <jats:styled-content style=\"fixed-case\">FAF<\/jats:styled-content> region was determined using one horizontal trans\u2010foveal <jats:styled-content style=\"fixed-case\">OCT<\/jats:styled-content> section.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Fourteen out of 20 patients had distinct smooth and mottled areas in both eyes and were included in the sub\u2010analysis. On average, 62.5\u00a0\u00b1\u00a010.1% of the <jats:styled-content style=\"fixed-case\">EZ<\/jats:styled-content> in each smooth region of the right eyes was intact compared to 10.0\u00a0\u00b1\u00a04.3% in the mottled areas. The same trend was observed in left eyes: 76.5\u00a0\u00b1\u00a07.2% of the <jats:styled-content style=\"fixed-case\">EZ<\/jats:styled-content> was intact in the smooth regions versus 9.8\u00a0\u00b1\u00a03.9% in the mottled areas (two\u2010way <jats:sc>anova<\/jats:sc>, p\u00a0&lt;\u00a00.0001). Thus, the mottled <jats:styled-content style=\"fixed-case\">FAF<\/jats:styled-content> regions were associated with <jats:styled-content style=\"fixed-case\">EZ<\/jats:styled-content> disruption more so than the smooth areas.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>Retinal pigment epithelium (<jats:styled-content style=\"fixed-case\">RPE<\/jats:styled-content>) changes correlate with the health of the overlying <jats:styled-content style=\"fixed-case\">EZ<\/jats:styled-content> in choroideremia. The smooth <jats:styled-content style=\"fixed-case\">FAF<\/jats:styled-content> region likely represents early stages of the disease, with most of the area containing preserved <jats:styled-content style=\"fixed-case\">EZ<\/jats:styled-content>, whereas the mottled zone indicates more advanced stages and has mostly disrupted <jats:styled-content style=\"fixed-case\">EZ<\/jats:styled-content>. Because of the clear relationship between <jats:styled-content style=\"fixed-case\">FAF<\/jats:styled-content> findings and <jats:styled-content style=\"fixed-case\">EZ<\/jats:styled-content> integrity, <jats:styled-content style=\"fixed-case\">FAF<\/jats:styled-content> imaging can be used to monitor disease progression and identify areas of preserved <jats:styled-content style=\"fixed-case\">EZ<\/jats:styled-content> that could be rescued by gene therapy.<\/jats:p><\/jats:sec>","DOI":"10.1111\/aos.14281","type":"journal-article","created":{"date-parts":[[2019,11,18]],"date-time":"2019-11-18T06:24:50Z","timestamp":1574058290000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":15,"title":["A distinct retinal pigment epithelial cell autofluorescence pattern in choroideremia predicts early involvement of overlying photoreceptors"],"prefix":"10.1111","volume":"98","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-6139-1373","authenticated-orcid":false,"given":"Marta","family":"Stevanovic","sequence":"first","affiliation":[{"name":"Nuffield Department of Clinical Neurosciences Nuffield Laboratory of Ophthalmology University of Oxford Oxford UK"},{"name":"Oxford Eye Hospital Oxford University Hospitals NHS Foundation Trust Oxford UK"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jasmina","family":"Cehajic Kapetanovic","sequence":"additional","affiliation":[{"name":"Nuffield Department of Clinical Neurosciences Nuffield Laboratory of Ophthalmology University of Oxford Oxford UK"},{"name":"Oxford Eye Hospital Oxford University Hospitals NHS Foundation Trust Oxford UK"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-9878-4621","authenticated-orcid":false,"given":"Jasleen K.","family":"Jolly","sequence":"additional","affiliation":[{"name":"Nuffield Department of Clinical Neurosciences Nuffield Laboratory of Ophthalmology University of Oxford Oxford UK"},{"name":"Oxford Eye Hospital Oxford University Hospitals NHS Foundation Trust Oxford UK"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-3096-4682","authenticated-orcid":false,"given":"Robert E.","family":"MacLaren","sequence":"additional","affiliation":[{"name":"Nuffield Department of Clinical Neurosciences Nuffield Laboratory of Ophthalmology University of Oxford Oxford UK"},{"name":"Oxford Eye Hospital Oxford University Hospitals NHS Foundation Trust Oxford 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