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Diego Garcia-Ayuso, Arturo Ortín-Martínez, Marta Agudo-Barriuso, Manuel Jiménez-López, Jose Manuel Bernal-Garro, Leticia Nieto-López, Nicolas Cuenca, Isabel Pinilla, Manuel Vidal-Sanz, María Paz Villegas-Pérez; Cone Degeneration In A Model Of Hereditary Rod Degeneration: The P23H Rat. Invest. Ophthalmol. Vis. Sci. 2011;52(14):4338.
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To examine the effect of the P23H rhodopsin mutation on S- and L- cones in the degenerating P23H transgenic rat retina.
P23H-1 homozygous albino rats at post-natal (P) day P30, P90 and P180 and age-matched albino Sprague-Dawley (SD) rats were examined. Whole-mounted retinas were incubated with antibodies against the S- and L- cones which differentiate three cone populations in the rat retina: the S-, L- and dual cones. The retinas were examined and photographed using a motorized fluorescence microscope and reconstructions of the whole mounts were obtained using Image-Pro Plus 5.0 for Windows®. The topography and the total numbers of the S- and L- cones were determined using an automatic counting method developed by our group (Ortín-Martínez et al., Invest. Ophthalmol. Vis. Sci. 51(2010): 3171-3183.
In the SD retina, the numbers and distribution of the S- and L- cones was similar to that observed in previous studies of our laboratory (Ortín-Martínez et al., Invest. Ophthalmol. Vis. Sci. 51(2010):3171-3183). In the homozygous P23H-1 rat retina, both types of cones exhibited shortened outer segments and abnormal opsin expression in their inner segment, soma, processes and synaptic terminals. Cone numbers decreased with time in the degenerating P23H retinas and their normal more or less even distribution was altered by the appearance of rosettes or ring-like shaped areas of cone degeneration, which increased with age.
The P23H rhodopsin mutation causes secondary cone degeneration. The degenerating cones exhibit changes in their morphology and opsin expression and altered spatial distribution. The spatial reorganization of cones into rosettes or ring-like shapes indicates differential cone cell death and deserves further investigation.
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