Schwann Cells in Neuromuscular Disorders: A Spotlight on Amyotrophic Lateral Sclerosis

Amyotrophic Lateral Sclerosis (ALS) is a complex neurodegenerative disease primarily affecting motor neurons, leading to progressive muscle atrophy and paralysis. This review explores the role of Schwann cells in ALS pathogenesis, highlighting their influence on disease progression through mechanism...

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Main Authors: Kathryn R. Moss, Smita Saxena
Format: Article
Language:English
Published: MDPI AG 2025-01-01
Series:Cells
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Online Access:https://www.mdpi.com/2073-4409/14/1/47
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author Kathryn R. Moss
Smita Saxena
author_facet Kathryn R. Moss
Smita Saxena
author_sort Kathryn R. Moss
collection DOAJ
description Amyotrophic Lateral Sclerosis (ALS) is a complex neurodegenerative disease primarily affecting motor neurons, leading to progressive muscle atrophy and paralysis. This review explores the role of Schwann cells in ALS pathogenesis, highlighting their influence on disease progression through mechanisms involving demyelination, neuroinflammation, and impaired synaptic function. While Schwann cells have been traditionally viewed as peripheral supportive cells, especially in motor neuron disease, recent evidence indicates that they play a significant role in ALS by impacting motor neuron survival and plasticity, influencing inflammatory responses, and altering myelination processes. Furthermore, advancements in understanding Schwann cell pathology in ALS combined with lessons learned from studying Charcot–Marie–Tooth disease Type 1 (CMT1) suggest potential therapeutic strategies targeting these cells may support nerve repair and slow disease progression. Overall, this review aims to provide comprehensive insights into Schwann cell classification, physiology, and function, underscoring the critical pathological contributions of Schwann cells in ALS and suggests new avenues for targeted therapeutic interventions aimed at modulating Schwann cell function in ALS.
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institution Kabale University
issn 2073-4409
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spelling doaj-art-685b6def18b644b3b506d4158586d4762025-01-10T13:16:21ZengMDPI AGCells2073-44092025-01-011414710.3390/cells14010047Schwann Cells in Neuromuscular Disorders: A Spotlight on Amyotrophic Lateral SclerosisKathryn R. Moss0Smita Saxena1Department of Physical Medicine and Rehabilitation, University of Missouri School of Medicine, Columbia, MO 65211, USADepartment of Physical Medicine and Rehabilitation, University of Missouri School of Medicine, Columbia, MO 65211, USAAmyotrophic Lateral Sclerosis (ALS) is a complex neurodegenerative disease primarily affecting motor neurons, leading to progressive muscle atrophy and paralysis. This review explores the role of Schwann cells in ALS pathogenesis, highlighting their influence on disease progression through mechanisms involving demyelination, neuroinflammation, and impaired synaptic function. While Schwann cells have been traditionally viewed as peripheral supportive cells, especially in motor neuron disease, recent evidence indicates that they play a significant role in ALS by impacting motor neuron survival and plasticity, influencing inflammatory responses, and altering myelination processes. Furthermore, advancements in understanding Schwann cell pathology in ALS combined with lessons learned from studying Charcot–Marie–Tooth disease Type 1 (CMT1) suggest potential therapeutic strategies targeting these cells may support nerve repair and slow disease progression. Overall, this review aims to provide comprehensive insights into Schwann cell classification, physiology, and function, underscoring the critical pathological contributions of Schwann cells in ALS and suggests new avenues for targeted therapeutic interventions aimed at modulating Schwann cell function in ALS.https://www.mdpi.com/2073-4409/14/1/47ALSCMT1Schwann cellmyelinterminal Schwann cellsNMJ
spellingShingle Kathryn R. Moss
Smita Saxena
Schwann Cells in Neuromuscular Disorders: A Spotlight on Amyotrophic Lateral Sclerosis
Cells
ALS
CMT1
Schwann cell
myelin
terminal Schwann cells
NMJ
title Schwann Cells in Neuromuscular Disorders: A Spotlight on Amyotrophic Lateral Sclerosis
title_full Schwann Cells in Neuromuscular Disorders: A Spotlight on Amyotrophic Lateral Sclerosis
title_fullStr Schwann Cells in Neuromuscular Disorders: A Spotlight on Amyotrophic Lateral Sclerosis
title_full_unstemmed Schwann Cells in Neuromuscular Disorders: A Spotlight on Amyotrophic Lateral Sclerosis
title_short Schwann Cells in Neuromuscular Disorders: A Spotlight on Amyotrophic Lateral Sclerosis
title_sort schwann cells in neuromuscular disorders a spotlight on amyotrophic lateral sclerosis
topic ALS
CMT1
Schwann cell
myelin
terminal Schwann cells
NMJ
url https://www.mdpi.com/2073-4409/14/1/47
work_keys_str_mv AT kathrynrmoss schwanncellsinneuromusculardisordersaspotlightonamyotrophiclateralsclerosis
AT smitasaxena schwanncellsinneuromusculardisordersaspotlightonamyotrophiclateralsclerosis