Frontiers in Cellular Neuroscience (Apr 2018)
Early Retinal Defects in Fmr1−/y Mice: Toward a Critical Role of Visual Dys-Sensitivity in the Fragile X Syndrome Phenotype?
- Olivier Perche,
- Olivier Perche,
- Olivier Perche,
- Chloé Felgerolle,
- Chloé Felgerolle,
- Maryvonne Ardourel,
- Maryvonne Ardourel,
- Audrey Bazinet,
- Audrey Bazinet,
- Arnaud Pâris,
- Arnaud Pâris,
- Rafaëlle Rossignol,
- Rafaëlle Rossignol,
- Géraldine Meyer-Dilhet,
- Géraldine Meyer-Dilhet,
- Anne-Laure Mausset-Bonnefont,
- Betty Hébert,
- Betty Hébert,
- David Laurenceau,
- Céline Montécot-Dubourg,
- Céline Montécot-Dubourg,
- Arnaud Menuet,
- Arnaud Menuet,
- Jean-Charles Bizot,
- Jacques Pichon,
- Jacques Pichon,
- Isabelle Ranchon-Cole,
- Sylvain Briault,
- Sylvain Briault,
- Sylvain Briault
Affiliations
- Olivier Perche
- Genetic Department, Centre Hospitalier Régional d’Orléans, Orléans, France
- Olivier Perche
- UMR7355, Immunologie et Neurogénétique Expérimentales et Moléculaires (INEM), Centre National de la Recherche Scientifique, Orléans, France
- Olivier Perche
- Experimental and Molecular Immunology and Neurogenetics, University of Orléans, Orléans, France
- Chloé Felgerolle
- UMR7355, Immunologie et Neurogénétique Expérimentales et Moléculaires (INEM), Centre National de la Recherche Scientifique, Orléans, France
- Chloé Felgerolle
- Experimental and Molecular Immunology and Neurogenetics, University of Orléans, Orléans, France
- Maryvonne Ardourel
- UMR7355, Immunologie et Neurogénétique Expérimentales et Moléculaires (INEM), Centre National de la Recherche Scientifique, Orléans, France
- Maryvonne Ardourel
- Experimental and Molecular Immunology and Neurogenetics, University of Orléans, Orléans, France
- Audrey Bazinet
- UMR7355, Immunologie et Neurogénétique Expérimentales et Moléculaires (INEM), Centre National de la Recherche Scientifique, Orléans, France
- Audrey Bazinet
- Experimental and Molecular Immunology and Neurogenetics, University of Orléans, Orléans, France
- Arnaud Pâris
- UMR7355, Immunologie et Neurogénétique Expérimentales et Moléculaires (INEM), Centre National de la Recherche Scientifique, Orléans, France
- Arnaud Pâris
- Experimental and Molecular Immunology and Neurogenetics, University of Orléans, Orléans, France
- Rafaëlle Rossignol
- UMR7355, Immunologie et Neurogénétique Expérimentales et Moléculaires (INEM), Centre National de la Recherche Scientifique, Orléans, France
- Rafaëlle Rossignol
- Experimental and Molecular Immunology and Neurogenetics, University of Orléans, Orléans, France
- Géraldine Meyer-Dilhet
- UMR7355, Immunologie et Neurogénétique Expérimentales et Moléculaires (INEM), Centre National de la Recherche Scientifique, Orléans, France
- Géraldine Meyer-Dilhet
- Experimental and Molecular Immunology and Neurogenetics, University of Orléans, Orléans, France
- Anne-Laure Mausset-Bonnefont
- IRMB, University of Montpellier, INSERM, Montpellier, France
- Betty Hébert
- UMR7355, Immunologie et Neurogénétique Expérimentales et Moléculaires (INEM), Centre National de la Recherche Scientifique, Orléans, France
- Betty Hébert
- Experimental and Molecular Immunology and Neurogenetics, University of Orléans, Orléans, France
- David Laurenceau
- Genetic Department, Centre Hospitalier Régional d’Orléans, Orléans, France
- Céline Montécot-Dubourg
- UMR7355, Immunologie et Neurogénétique Expérimentales et Moléculaires (INEM), Centre National de la Recherche Scientifique, Orléans, France
- Céline Montécot-Dubourg
- Experimental and Molecular Immunology and Neurogenetics, University of Orléans, Orléans, France
- Arnaud Menuet
- UMR7355, Immunologie et Neurogénétique Expérimentales et Moléculaires (INEM), Centre National de la Recherche Scientifique, Orléans, France
- Arnaud Menuet
- Experimental and Molecular Immunology and Neurogenetics, University of Orléans, Orléans, France
- Jean-Charles Bizot
- KeyObs, CRO Pharmacology, Orléans, France
- Jacques Pichon
- UMR7355, Immunologie et Neurogénétique Expérimentales et Moléculaires (INEM), Centre National de la Recherche Scientifique, Orléans, France
- Jacques Pichon
- Experimental and Molecular Immunology and Neurogenetics, University of Orléans, Orléans, France
- Isabelle Ranchon-Cole
- Laboratory of Sensorial Biophysical, INSERM UMR1107 Equipe Biophysique Neurosensorielle, University of Clermont 1, Clermont-Ferrand, France
- Sylvain Briault
- Genetic Department, Centre Hospitalier Régional d’Orléans, Orléans, France
- Sylvain Briault
- UMR7355, Immunologie et Neurogénétique Expérimentales et Moléculaires (INEM), Centre National de la Recherche Scientifique, Orléans, France
- Sylvain Briault
- Experimental and Molecular Immunology and Neurogenetics, University of Orléans, Orléans, France
- DOI
- https://doi.org/10.3389/fncel.2018.00096
- Journal volume & issue
-
Vol. 12
Abstract
Fragile X Syndrome (FXS) is caused by a deficiency in Fragile X Mental Retardation Protein (FMRP) leading to global sensorial abnormalities, among which visual defects represent a critical part. These visual defects are associated with cerebral neuron immaturity especially in the primary visual cortex. However, we recently demonstrated that retinas of adult Fmr1−/y mice, the FXS murine model, present molecular, cellular and functional alterations. However, no data are currently available on the evolution pattern of such defects. As retinal stimulation through Eye Opening (EO) is a crucial signal for the cerebral visual system maturation, we questioned the precocity of molecular and functional retinal phenotype. To answer this question, we studied the retinal molecular phenotype of Fmr1−/y mice before EO until adult age and the consequences of the retinal loss of Fmrp on retinal function in young and adult mice. We showed that retinal molecular defects are present before EO and remain stable at adult age, leading to electrophysiological impairments without any underlying structural changes. We underlined that loss of Fmrp leads to a wide range of defects in the retina, settled even before EO. Our work demonstrates a critical role of the sensorial dysfunction in the Fmr1−/y mice overall phenotype, and provides evidence that altered peripheral perception is a component of the sensory processing defect in FXS conditions.
Keywords