Early Events in Retinal Degeneration Caused by Rhodopsin Mutation or Pigment Epithelium Malfunction: Differences and Similarities

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Title: Early Events in Retinal Degeneration Caused by Rhodopsin Mutation or Pigment Epithelium Malfunction: Differences and Similarities
Authors: Di Pierdomenico, Johnny | García Ayuso, Diego | Pinilla Lozano, Isabel | Cuenca, Nicolás | Vidal Sanz, Manuel | Agudo Barriuso, Marta | Villegas Pérez, María Paz
Research Group/s: Neurobiología del Sistema Visual y Terapia de Enfermedades Neurodegenerativas (NEUROVIS)
Center, Department or Service: Universidad de Alicante. Departamento de Fisiología, Genética y Microbiología
Keywords: Retina | Inherited retinal degeneration | Royal College of Surgeons | P23H-1 | Microglia | Glial fibrillary acidic protein | Müller cells | Rat
Knowledge Area: Biología Celular
Issue Date: 6-Mar-2017
Publisher: Frontiers Media
Citation: Di Pierdomenico J, García-Ayuso D, Pinilla I, Cuenca N, Vidal-Sanz M, Agudo-Barriuso M and Villegas-Pérez MP (2017) Early Events in Retinal Degeneration Caused by Rhodopsin Mutation or Pigment Epithelium Malfunction: Differences and Similarities. Front. Neuroanat. 11:14. doi: 10.3389/fnana.2017.00014
Abstract: To study the course of photoreceptor cell death and macro and microglial reactivity in two rat models of retinal degeneration with different etiologies. Retinas from P23H-1 (rhodopsin mutation) and Royal College of Surgeon (RCS, pigment epithelium malfunction) rats and age-matched control animals (Sprague-Dawley and Pievald Viro Glaxo, respectively) were cross-sectioned at different postnatal ages (from P10 to P60) and rhodopsin, L/M- and S-opsin, ionized calcium-binding adapter molecule 1 (Iba1), glial fibrillary acid protein (GFAP), and proliferating cell nuclear antigen (PCNA) proteins were immunodetected. Photoreceptor nuclei rows and microglial cells in the different retinal layers were quantified. Photoreceptor degeneration starts earlier and progresses quicker in P23H-1 than in RCS rats. In both models, microglial cell activation occurs simultaneously with the initiation of photoreceptor death while GFAP over-expression starts later. As degeneration progresses, the numbers of microglial cells increase in the retina, but decreasing in the inner retina and increasing in the outer retina, more markedly in RCS rats. Interestingly, and in contrast with healthy animals, microglial cells reach the outer nuclei and outer segment layers. The higher number of microglial cells in dystrophic retinas cannot be fully accounted by intraretinal migration and PCNA immunodetection revealed microglial proliferation in both models but more importantly in RCS rats. The etiology of retinal degeneration determines the initiation and pattern of photoreceptor cell death and simultaneously there is microglial activation and migration, while the macroglial response is delayed. The actions of microglial cells in the degeneration cannot be explained only in the basis of photoreceptor death because they participate more actively in the RCS model. Thus, the retinal degeneration caused by pigment epithelium malfunction is more inflammatory and would probably respond better to interventions by inhibiting microglial cells.
Sponsor: Fundación Séneca, Agencia de Ciencia y Tecnología Región de Murcia (19881/GERM/15) and the Spanish Ministry of Economy and Competitiveness, Instituto de Salud Carlos III, Fondo Europeo de Desarrollo Regional “Una Manera de Hacer Europa” ISCIII-FEDER PI16/00380, PI16/00031, RD16/0008/0026, RD16/0008/0016, SAF2015-67643.
URI: http://hdl.handle.net/10045/64368
ISSN: 1662-5129
DOI: 10.3389/fnana.2017.00014
Language: eng
Type: info:eu-repo/semantics/article
Rights: © 2017 Di Pierdomenico, García-Ayuso, Pinilla, Cuenca, Vidal-Sanz, Agudo-Barriuso and Villegas-Pérez. This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) or licensor are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.
Peer Review: si
Publisher version: http://dx.doi.org/10.3389/fnana.2017.00014
Appears in Collections:INV - NEUROVIS - Artículos de Revistas

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