{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,1,3]],"date-time":"2025-01-03T05:40:52Z","timestamp":1735882852110,"version":"3.32.0"},"reference-count":62,"publisher":"Maximum Academic Press","issue":"6","license":[{"start":{"date-parts":[[2006,2,13]],"date-time":"2006-02-13T00:00:00Z","timestamp":1139788800000},"content-version":"unspecified","delay-in-days":104,"URL":"https:\/\/www.cambridge.org\/core\/terms"}],"content-domain":{"domain":["journals.cambridge.org"],"crossmark-restriction":true},"short-container-title":["Vis Neurosci"],"published-print":{"date-parts":[[2005,11]]},"abstract":"<jats:p>Retinal development in 3 strains of <jats:italic>rd-3\/rd-3<\/jats:italic> mutant \nmice, previously shown to have different rates of degeneration, was \nstudied using light, electron, and immunofluorescence microscopy. The time \ncourse and phenotype of the degeneration as well as details on the \nmechanism of massive photoreceptor cell loss are compared with other known \nretinal degenerations in mice. Up until postnatal day (P) 10, the retinas \nof all three strains (RBF, 4Bnr, In-30) develop similarly to those of \npigmented and nonpigmented controls. TUNEL-positive cells appear in the \nouter nuclear layer (ONL) by P14, and reach a maximum in all three mutant \nstrains around P21. Scattered rods and cones form a loose, monolayered ONL \nby 8 weeks in the albino RBF strain, by 10 weeks in the albino 4Bnr \nstrain, and by 16 weeks in the pigmented In-30 strain. Though the initial \ndegeneration begins in the central retina, there is no preferred gradient \nof cell death between central and peripheral photoreceptors. Rods and \ncones are present at all ages examined. During development, stacks of \nouter segments (OS) form in all three strains though they never achieve \nfull adult lengths, and often have disorganized, atypical OS. Rod opsin is \nexpressed in the developing OS but is redistributed into plasma membrane \nas OS degeneration proceeds. Retinal pigment epithelial (RPE) cells of all \nmutant strains contain packets of phagocytosed OS, and their apical \nprocesses associate with the distal ends of the OS. At their synaptic \nsites, photoreceptor terminals contain ribbons apposed to apparently \nnormal postsynaptic triads. As photoreceptors are lost, M\u00fcller cells \nfill in space in the ONL but they do not appear to undergo significant \nhypertrophy or migration, though during the degeneration, glial fibrillary \nacidic protein (GFAP) expression is gradually upregulated. Macrophage-like \ncells are found frequently in the subretinal space after the onset of \nphotoreceptor apoptosis. As OS disappear, the RPE apical processes revert \nto simple microvilli. Late in the degeneration, some RPE cells die and \nneighboring cells appear to flatten as if to maintain confluence. In \nregions of RPE cell loss that happen to lie above retina where the ONL is \ngone, cells of the inner nuclear layer (INL), wrapped by M\u00fcller cell \nprocesses, may front directly on Bruch's membrane.<\/jats:p>","DOI":"10.1017\/s0952523805226044","type":"journal-article","created":{"date-parts":[[2008,4,21]],"date-time":"2008-04-21T18:10:21Z","timestamp":1208801421000},"page":"721-734","update-policy":"https:\/\/doi.org\/10.1017\/policypage","source":"Crossref","is-referenced-by-count":15,"title":["Morphological characterization of the retinal degeneration in three \nstrains of mice carrying the <i>rd-3<\/i> mutation"],"prefix":"10.48130","volume":"22","author":[{"given":"KENNETH A.","family":"LINBERG","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"ROBERT N.","family":"FARISS","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"JOHN R.","family":"HECKENLIVELY","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"DEBORA B.","family":"FARBER","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"STEVEN K.","family":"FISHER","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"27968","published-online":{"date-parts":[[2006,2,13]]},"reference":[{"key":"S0952523805226044_ref059","doi-asserted-by":"crossref","unstructured":"Strettoi, E. & Pignatelli, V. 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