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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2025-01-01
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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. |
format | Article |
id | doaj-art-685b6def18b644b3b506d4158586d476 |
institution | Kabale University |
issn | 2073-4409 |
language | English |
publishDate | 2025-01-01 |
publisher | MDPI AG |
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series | Cells |
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 |