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...
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doaj-35c56c7f0ac84fef9ec415922907fe872020-11-25T02:43:09ZengMDPI AGInternational Journal of Molecular Sciences1422-00672017-10-011810209210.3390/ijms18102092ijms18102092Neuromuscular Junction Dismantling in Amyotrophic Lateral SclerosisValentina Cappello0Maura Francolini1Center for Nanotechnology Innovation@NEST, Istituto Italiano di Tecnologia Piazza San Silvestro 12, 56127 Pisa, ItalyDepartment of Medical Biotechnology and Translational Medicine, Università degli Studi di Milano—Via Vanvitelli 32, 20129 Milano, ItalyNeuromuscular 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.https://www.mdpi.com/1422-0067/18/10/2092neuromuscular junctionAmyotrophic Lateral Sclerosistripartite synapse |
collection |
DOAJ |
language |
English |
format |
Article |
sources |
DOAJ |
author |
Valentina Cappello Maura Francolini |
spellingShingle |
Valentina Cappello Maura Francolini Neuromuscular Junction Dismantling in Amyotrophic Lateral Sclerosis International Journal of Molecular Sciences neuromuscular junction Amyotrophic Lateral Sclerosis tripartite synapse |
author_facet |
Valentina Cappello Maura Francolini |
author_sort |
Valentina Cappello |
title |
Neuromuscular Junction Dismantling in Amyotrophic Lateral Sclerosis |
title_short |
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_sort |
neuromuscular junction dismantling in amyotrophic lateral sclerosis |
publisher |
MDPI AG |
series |
International Journal of Molecular Sciences |
issn |
1422-0067 |
publishDate |
2017-10-01 |
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. |
topic |
neuromuscular junction Amyotrophic Lateral Sclerosis tripartite synapse |
url |
https://www.mdpi.com/1422-0067/18/10/2092 |
work_keys_str_mv |
AT valentinacappello neuromuscularjunctiondismantlinginamyotrophiclateralsclerosis AT maurafrancolini neuromuscularjunctiondismantlinginamyotrophiclateralsclerosis |
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1724771164414279680 |