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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...

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Autores principales: Crabé, Roxane, Aimond, Franck, Gosset, Philippe, Scamps, Frédérique, Raoul, Cédric
Formato: Online Artículo Texto
Lenguaje:English
Publicado: MDPI 2020
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.
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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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