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We characterized in detail C57BL\/6J <jats:italic>Nr2e3<\/jats:italic><jats:sup><jats:italic>rd7\/rd7<\/jats:italic><\/jats:sup> mice in vivo by fundus photography, optical coherence tomography and fluorescein angiography and, post mortem, by histology and immunohistochemistry. White retinal spots and so-called \u2018rosettes\u2019 first appear at postnatal day (P) 12 in the dorsal retina and reach maximal expansion at P21. The highest density in \u2018rosettes\u2019 is observed within a region located between 100 and 350\u00a0\u00b5M from the optic nerve head. \u2018Rosettes\u2019 disappear between 9 to 12\u00a0months. Non-apoptotic cell death markers are detected during the slow photoreceptor degeneration, at a rate of an approximately 3% reduction of outer nuclear layer thickness per month, as observed from 7 to 31\u00a0months of age. In vivo analysis of <jats:italic>Nr2e3<\/jats:italic><jats:sup><jats:italic>rd7\/rd7<\/jats:italic><\/jats:sup><jats:italic> Cx3cr1<\/jats:italic><jats:sup><jats:italic>gfp\/<\/jats:italic>+<\/jats:sup> retinas identified microglial cells within \u2018rosettes\u2019 from P21 on. Subretinal macrophages were observed in vivo and by confocal microscopy earliest in 12-months-old <jats:italic>Nr2e3<\/jats:italic><jats:sup><jats:italic>rd7\/rd7<\/jats:italic><\/jats:sup> retinas. At P21, S-opsin expression and the number of S-opsin expressing dorsal cones was increased. The dorso-ventral M-cone gradient was present in <jats:italic>Nr2e3<\/jats:italic><jats:sup><jats:italic>rd7\/rd7<\/jats:italic><\/jats:sup> retinas, but M-opsin expression and M-opsin expressing cones were decreased. Retinal vasculature was normal.<\/jats:p>","DOI":"10.1038\/s41598-021-98271-7","type":"journal-article","created":{"date-parts":[[2021,9,29]],"date-time":"2021-09-29T20:06:05Z","timestamp":1632945965000},"update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":6,"title":["In vivo analysis of onset and progression of retinal degeneration in the Nr2e3rd7\/rd7 mouse model of enhanced S-cone sensitivity syndrome"],"prefix":"10.1038","volume":"11","author":[{"given":"Giulia","family":"Venturini","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Despina","family":"Kokona","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Beatrice L.","family":"Steiner","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Emanuele G.","family":"Bulla","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-9195-2239","authenticated-orcid":false,"given":"Joel","family":"Jovanovic","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-3447-2359","authenticated-orcid":false,"given":"Martin S.","family":"Zinkernagel","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-8281-2875","authenticated-orcid":false,"given":"Pascal","family":"Escher","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"297","published-online":{"date-parts":[[2021,9,24]]},"reference":[{"key":"98271_CR1","doi-asserted-by":"publisher","first-page":"2146","DOI":"10.1093\/hmg\/ddl138","volume":"15","author":"J Chen","year":"2006","unstructured":"Chen, J., Rattner, A. & Nathans, J. 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