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How Degeneration of Cells Surrounding Motoneurons Contributes to Amyotrophic Lateral Sclerosis
Amyotrophic lateral sclerosis (ALS) is a fatal neurological disorder characterized by the progressive degeneration of upper and lower motoneurons. Despite motoneuron death being recognized as the cardinal event of the disease, the loss of glial cells and interneurons in the brain and spinal cord acc...
Autores principales: | , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
MDPI
2020
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7760029/ https://www.ncbi.nlm.nih.gov/pubmed/33260927 http://dx.doi.org/10.3390/cells9122550 |
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author | Crabé, Roxane Aimond, Franck Gosset, Philippe Scamps, Frédérique Raoul, Cédric |
author_facet | Crabé, Roxane Aimond, Franck Gosset, Philippe Scamps, Frédérique Raoul, Cédric |
author_sort | Crabé, Roxane |
collection | PubMed |
description | Amyotrophic lateral sclerosis (ALS) is a fatal neurological disorder characterized by the progressive degeneration of upper and lower motoneurons. Despite motoneuron death being recognized as the cardinal event of the disease, the loss of glial cells and interneurons in the brain and spinal cord accompanies and even precedes motoneuron elimination. In this review, we provide striking evidence that the degeneration of astrocytes and oligodendrocytes, in addition to inhibitory and modulatory interneurons, disrupt the functionally coherent environment of motoneurons. We discuss the extent to which the degeneration of glial cells and interneurons also contributes to the decline of the motor system. This pathogenic cellular network therefore represents a novel strategic field of therapeutic investigation. |
format | Online Article Text |
id | pubmed-7760029 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2020 |
publisher | MDPI |
record_format | MEDLINE/PubMed |
spelling | pubmed-77600292020-12-26 How Degeneration of Cells Surrounding Motoneurons Contributes to Amyotrophic Lateral Sclerosis Crabé, Roxane Aimond, Franck Gosset, Philippe Scamps, Frédérique Raoul, Cédric Cells Review Amyotrophic lateral sclerosis (ALS) is a fatal neurological disorder characterized by the progressive degeneration of upper and lower motoneurons. Despite motoneuron death being recognized as the cardinal event of the disease, the loss of glial cells and interneurons in the brain and spinal cord accompanies and even precedes motoneuron elimination. In this review, we provide striking evidence that the degeneration of astrocytes and oligodendrocytes, in addition to inhibitory and modulatory interneurons, disrupt the functionally coherent environment of motoneurons. We discuss the extent to which the degeneration of glial cells and interneurons also contributes to the decline of the motor system. This pathogenic cellular network therefore represents a novel strategic field of therapeutic investigation. MDPI 2020-11-27 /pmc/articles/PMC7760029/ /pubmed/33260927 http://dx.doi.org/10.3390/cells9122550 Text en © 2020 by the authors. 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 (http://creativecommons.org/licenses/by/4.0/). |
spellingShingle | Review Crabé, Roxane Aimond, Franck Gosset, Philippe Scamps, Frédérique Raoul, Cédric How Degeneration of Cells Surrounding Motoneurons Contributes to Amyotrophic Lateral Sclerosis |
title | How Degeneration of Cells Surrounding Motoneurons Contributes to Amyotrophic Lateral Sclerosis |
title_full | How Degeneration of Cells Surrounding Motoneurons Contributes to Amyotrophic Lateral Sclerosis |
title_fullStr | How Degeneration of Cells Surrounding Motoneurons Contributes to Amyotrophic Lateral Sclerosis |
title_full_unstemmed | How Degeneration of Cells Surrounding Motoneurons Contributes to Amyotrophic Lateral Sclerosis |
title_short | How Degeneration of Cells Surrounding Motoneurons Contributes to Amyotrophic Lateral Sclerosis |
title_sort | how degeneration of cells surrounding motoneurons contributes to amyotrophic lateral sclerosis |
topic | Review |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7760029/ https://www.ncbi.nlm.nih.gov/pubmed/33260927 http://dx.doi.org/10.3390/cells9122550 |
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