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Visual Cortex Engagement in Retinitis Pigmentosa

Retinitis pigmentosa (RP) is a family of inherited disorders caused by the progressive degeneration of retinal photoreceptors. There is no cure for RP, but recent research advances have provided promising results from many clinical trials. All these therapeutic strategies are focused on preserving e...

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Autores principales: Pietra, Gianluca, Bonifacino, Tiziana, Talamonti, Davide, Bonanno, Giambattista, Sale, Alessandro, Galli, Lucia, Baroncelli, Laura
Formato: Online Artículo Texto
Lenguaje:English
Publicado: MDPI 2021
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8431500/
https://www.ncbi.nlm.nih.gov/pubmed/34502320
http://dx.doi.org/10.3390/ijms22179412
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author Pietra, Gianluca
Bonifacino, Tiziana
Talamonti, Davide
Bonanno, Giambattista
Sale, Alessandro
Galli, Lucia
Baroncelli, Laura
author_facet Pietra, Gianluca
Bonifacino, Tiziana
Talamonti, Davide
Bonanno, Giambattista
Sale, Alessandro
Galli, Lucia
Baroncelli, Laura
author_sort Pietra, Gianluca
collection PubMed
description Retinitis pigmentosa (RP) is a family of inherited disorders caused by the progressive degeneration of retinal photoreceptors. There is no cure for RP, but recent research advances have provided promising results from many clinical trials. All these therapeutic strategies are focused on preserving existing photoreceptors or substituting light-responsive elements. Vision recovery, however, strongly relies on the anatomical and functional integrity of the visual system beyond photoreceptors. Although the retinal structure and optic pathway are substantially preserved at least in early stages of RP, studies describing the visual cortex status are missing. Using a well-established mouse model of RP, we analyzed the response of visual cortical circuits to the progressive degeneration of photoreceptors. We demonstrated that the visual cortex goes through a transient and previously undescribed alteration in the local excitation/inhibition balance, with a net shift towards increased intracortical inhibition leading to improved filtering and decoding of corrupted visual inputs. These results suggest a compensatory action of the visual cortex that increases the range of residual visual sensitivity in RP.
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spelling pubmed-84315002021-09-11 Visual Cortex Engagement in Retinitis Pigmentosa Pietra, Gianluca Bonifacino, Tiziana Talamonti, Davide Bonanno, Giambattista Sale, Alessandro Galli, Lucia Baroncelli, Laura Int J Mol Sci Article Retinitis pigmentosa (RP) is a family of inherited disorders caused by the progressive degeneration of retinal photoreceptors. There is no cure for RP, but recent research advances have provided promising results from many clinical trials. All these therapeutic strategies are focused on preserving existing photoreceptors or substituting light-responsive elements. Vision recovery, however, strongly relies on the anatomical and functional integrity of the visual system beyond photoreceptors. Although the retinal structure and optic pathway are substantially preserved at least in early stages of RP, studies describing the visual cortex status are missing. Using a well-established mouse model of RP, we analyzed the response of visual cortical circuits to the progressive degeneration of photoreceptors. We demonstrated that the visual cortex goes through a transient and previously undescribed alteration in the local excitation/inhibition balance, with a net shift towards increased intracortical inhibition leading to improved filtering and decoding of corrupted visual inputs. These results suggest a compensatory action of the visual cortex that increases the range of residual visual sensitivity in RP. MDPI 2021-08-30 /pmc/articles/PMC8431500/ /pubmed/34502320 http://dx.doi.org/10.3390/ijms22179412 Text en © 2021 by the authors. https://creativecommons.org/licenses/by/4.0/Licensee MDPI, Basel, Switzerland. This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution (CC BY) license (https://creativecommons.org/licenses/by/4.0/).
spellingShingle Article
Pietra, Gianluca
Bonifacino, Tiziana
Talamonti, Davide
Bonanno, Giambattista
Sale, Alessandro
Galli, Lucia
Baroncelli, Laura
Visual Cortex Engagement in Retinitis Pigmentosa
title Visual Cortex Engagement in Retinitis Pigmentosa
title_full Visual Cortex Engagement in Retinitis Pigmentosa
title_fullStr Visual Cortex Engagement in Retinitis Pigmentosa
title_full_unstemmed Visual Cortex Engagement in Retinitis Pigmentosa
title_short Visual Cortex Engagement in Retinitis Pigmentosa
title_sort visual cortex engagement in retinitis pigmentosa
topic Article
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8431500/
https://www.ncbi.nlm.nih.gov/pubmed/34502320
http://dx.doi.org/10.3390/ijms22179412
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