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Neuromuscular Junction Dismantling in Amyotrophic Lateral Sclerosis
Neuromuscular junction assembly and plasticity during embryonic, postnatal, and adult life are tightly regulated by the continuous cross-talk among motor nerve endings, muscle fibers, and glial cells. Altered communications among these components is thought to be responsible for the physiological ag...
Autores principales: | , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
MDPI
2017
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5666774/ https://www.ncbi.nlm.nih.gov/pubmed/28972545 http://dx.doi.org/10.3390/ijms18102092 |
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author | Cappello, Valentina Francolini, Maura |
author_facet | Cappello, Valentina Francolini, Maura |
author_sort | Cappello, Valentina |
collection | PubMed |
description | Neuromuscular junction assembly and plasticity during embryonic, postnatal, and adult life are tightly regulated by the continuous cross-talk among motor nerve endings, muscle fibers, and glial cells. Altered communications among these components is thought to be responsible for the physiological age-related changes at this synapse and possibly for its destruction in pathological states. Neuromuscular junction dismantling plays a crucial role in the onset of Amyotrophic Lateral Sclerosis (ALS). ALS is characterized by the degeneration and death of motor neurons leading to skeletal muscle denervation, atrophy and, most often, death of the patient within five years from diagnosis. ALS is a non-cell autonomous disease as, besides motor neuron degeneration, glial cells, and possibly muscle fibers, play a role in its onset and progression. Here, we will review the recent literature regarding the mechanisms leading to neuromuscular junction disassembly and muscle denervation focusing on the role of the three players of this peripheral tripartite synapse. |
format | Online Article Text |
id | pubmed-5666774 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2017 |
publisher | MDPI |
record_format | MEDLINE/PubMed |
spelling | pubmed-56667742017-11-09 Neuromuscular Junction Dismantling in Amyotrophic Lateral Sclerosis Cappello, Valentina Francolini, Maura Int J Mol Sci Review Neuromuscular junction assembly and plasticity during embryonic, postnatal, and adult life are tightly regulated by the continuous cross-talk among motor nerve endings, muscle fibers, and glial cells. Altered communications among these components is thought to be responsible for the physiological age-related changes at this synapse and possibly for its destruction in pathological states. Neuromuscular junction dismantling plays a crucial role in the onset of Amyotrophic Lateral Sclerosis (ALS). ALS is characterized by the degeneration and death of motor neurons leading to skeletal muscle denervation, atrophy and, most often, death of the patient within five years from diagnosis. ALS is a non-cell autonomous disease as, besides motor neuron degeneration, glial cells, and possibly muscle fibers, play a role in its onset and progression. Here, we will review the recent literature regarding the mechanisms leading to neuromuscular junction disassembly and muscle denervation focusing on the role of the three players of this peripheral tripartite synapse. MDPI 2017-10-03 /pmc/articles/PMC5666774/ /pubmed/28972545 http://dx.doi.org/10.3390/ijms18102092 Text en © 2017 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 Cappello, Valentina Francolini, Maura Neuromuscular Junction Dismantling in Amyotrophic Lateral Sclerosis |
title | Neuromuscular Junction Dismantling in Amyotrophic Lateral Sclerosis |
title_full | Neuromuscular Junction Dismantling in Amyotrophic Lateral Sclerosis |
title_fullStr | Neuromuscular Junction Dismantling in Amyotrophic Lateral Sclerosis |
title_full_unstemmed | Neuromuscular Junction Dismantling in Amyotrophic Lateral Sclerosis |
title_short | Neuromuscular Junction Dismantling in Amyotrophic Lateral Sclerosis |
title_sort | neuromuscular junction dismantling in amyotrophic lateral sclerosis |
topic | Review |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5666774/ https://www.ncbi.nlm.nih.gov/pubmed/28972545 http://dx.doi.org/10.3390/ijms18102092 |
work_keys_str_mv | AT cappellovalentina neuromuscularjunctiondismantlinginamyotrophiclateralsclerosis AT francolinimaura neuromuscularjunctiondismantlinginamyotrophiclateralsclerosis |