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神经肌肉疾病中的施万细胞:聚焦肌萎缩侧索硬化症

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

作者信息

Moss Kathryn R, Saxena Smita

机构信息

Department of Physical Medicine and Rehabilitation, University of Missouri School of Medicine, Columbia, MO 65211, USA.

NextGen Precision Health, University of Missouri, Columbia, MO 65211, USA.

出版信息

Cells. 2025 Jan 3;14(1):47. doi: 10.3390/cells14010047.

DOI:10.3390/cells14010047
PMID:39791748
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC11719703/
Abstract

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.

摘要

肌萎缩侧索硬化症(ALS)是一种复杂的神经退行性疾病,主要影响运动神经元,导致进行性肌肉萎缩和瘫痪。本综述探讨了雪旺细胞在ALS发病机制中的作用,强调了它们通过脱髓鞘、神经炎症和突触功能受损等机制对疾病进展的影响。虽然雪旺细胞传统上被视为外周支持细胞,尤其是在运动神经元疾病中,但最近的证据表明,它们通过影响运动神经元的存活和可塑性、影响炎症反应以及改变髓鞘形成过程,在ALS中发挥着重要作用。此外,对ALS中雪旺细胞病理学的理解进展以及从研究1型夏科-马里-图思病(CMT1)中获得的经验教训表明,针对这些细胞的潜在治疗策略可能有助于神经修复并减缓疾病进展。总体而言,本综述旨在全面深入了解雪旺细胞的分类、生理学和功能,强调雪旺细胞在ALS中的关键病理贡献,并提出针对ALS中调节雪旺细胞功能的靶向治疗干预的新途径。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/658a/11719703/327d57760a93/cells-14-00047-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/658a/11719703/327d57760a93/cells-14-00047-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/658a/11719703/327d57760a93/cells-14-00047-g001.jpg

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Perisynaptic Schwann Cells: Guardians of Neuromuscular Junction Integrity and Function in Health and Disease.突触周围施万细胞:健康与疾病状态下神经肌肉接头完整性及功能的守护者
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Pathologic TDP-43 downregulates myelin gene expression in the monkey brain.
病理性 TDP-43 下调猴脑髓鞘基因表达。
Brain Pathol. 2024 Nov;34(6):e13277. doi: 10.1111/bpa.13277. Epub 2024 May 23.
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Origin, identity, and function of terminal Schwann cells.终端许旺细胞的起源、特性和功能。
Trends Neurosci. 2024 Jun;47(6):432-446. doi: 10.1016/j.tins.2024.03.007. Epub 2024 Apr 24.
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