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Degeneration-Dependent Retinal Remodeling: Looking for the Molecular Trigger

Vision impairment and blindness in humans are most frequently caused by the degeneration and loss of photoreceptor cells in the outer retina, as is the case for age-related macular degeneration, retinitis pigmentosa, retinal detachment and many other diseases. While inner retinal neurons survive deg...

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Autores principales: Telias, Michael, Nawy, Scott, Kramer, Richard H.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Frontiers Media S.A. 2020
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7775662/
https://www.ncbi.nlm.nih.gov/pubmed/33390897
http://dx.doi.org/10.3389/fnins.2020.618019
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author Telias, Michael
Nawy, Scott
Kramer, Richard H.
author_facet Telias, Michael
Nawy, Scott
Kramer, Richard H.
author_sort Telias, Michael
collection PubMed
description Vision impairment and blindness in humans are most frequently caused by the degeneration and loss of photoreceptor cells in the outer retina, as is the case for age-related macular degeneration, retinitis pigmentosa, retinal detachment and many other diseases. While inner retinal neurons survive degeneration, they undergo fundamental pathophysiological changes, collectively known as “remodeling.” Inner retinal remodeling downstream to photoreceptor death occurs across mammalian retinas from mice to humans, independently of the cause of degeneration. It results in pervasive spontaneous hyperactivity and membrane hyperpermeability in retinal ganglion cells, which funnel all retinal signals to the brain. Remodeling reduces light detection in vision-impaired patients and precludes meaningful vision restoration in blind individuals. In this review, we summarize current hypotheses proposed to explain remodeling and their potential medical significance highlighting the important role played by retinoic acid and its receptor.
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spelling pubmed-77756622021-01-02 Degeneration-Dependent Retinal Remodeling: Looking for the Molecular Trigger Telias, Michael Nawy, Scott Kramer, Richard H. Front Neurosci Neuroscience Vision impairment and blindness in humans are most frequently caused by the degeneration and loss of photoreceptor cells in the outer retina, as is the case for age-related macular degeneration, retinitis pigmentosa, retinal detachment and many other diseases. While inner retinal neurons survive degeneration, they undergo fundamental pathophysiological changes, collectively known as “remodeling.” Inner retinal remodeling downstream to photoreceptor death occurs across mammalian retinas from mice to humans, independently of the cause of degeneration. It results in pervasive spontaneous hyperactivity and membrane hyperpermeability in retinal ganglion cells, which funnel all retinal signals to the brain. Remodeling reduces light detection in vision-impaired patients and precludes meaningful vision restoration in blind individuals. In this review, we summarize current hypotheses proposed to explain remodeling and their potential medical significance highlighting the important role played by retinoic acid and its receptor. Frontiers Media S.A. 2020-12-18 /pmc/articles/PMC7775662/ /pubmed/33390897 http://dx.doi.org/10.3389/fnins.2020.618019 Text en Copyright © 2020 Telias, Nawy and Kramer. http://creativecommons.org/licenses/by/4.0/ 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) and the copyright owner(s) 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.
spellingShingle Neuroscience
Telias, Michael
Nawy, Scott
Kramer, Richard H.
Degeneration-Dependent Retinal Remodeling: Looking for the Molecular Trigger
title Degeneration-Dependent Retinal Remodeling: Looking for the Molecular Trigger
title_full Degeneration-Dependent Retinal Remodeling: Looking for the Molecular Trigger
title_fullStr Degeneration-Dependent Retinal Remodeling: Looking for the Molecular Trigger
title_full_unstemmed Degeneration-Dependent Retinal Remodeling: Looking for the Molecular Trigger
title_short Degeneration-Dependent Retinal Remodeling: Looking for the Molecular Trigger
title_sort degeneration-dependent retinal remodeling: looking for the molecular trigger
topic Neuroscience
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7775662/
https://www.ncbi.nlm.nih.gov/pubmed/33390897
http://dx.doi.org/10.3389/fnins.2020.618019
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