Chen Xiaosu, Lv Shuchang, Liu Jinmeng, Guan Yingjun, Xu Chunjie, Ma Xiaonan, Li Mu, Bai Xue, Liu Kexin, Zhang Haoyun, Yan Qiupeng, Zhou Fenghua, Chen Yanchun
Department of Histology and Embryology, Shandong Second Medical University, Weifang 261053, China.
Neurologic Disorders and Regenerative Repair Lab of Shandong Higher Education, Shandong Second Medical University, Weifang 261053, China.
Cells. 2024 Dec 17;13(24):2076. doi: 10.3390/cells13242076.
Amyotrophic lateral sclerosis (ALS), commonly known as motor neuron disease, is a neurodegenerative disorder characterized by the progressive degeneration of both upper and lower motor neurons. This pathological process results in muscle weakness and can culminate in paralysis. To date, the precise etiology of ALS remains unclear. However, a burgeoning body of research indicates that axonal dysfunction is a pivotal element in the pathogenesis of ALS and significantly influences the progression of disease. Dysfunction of axons in ALS can result in impediments to nerve impulse transmission, leading to motor impairment, muscle atrophy, and other associated complications that severely compromise patients' quality of life and survival prognosis. In this review, we concentrate on several key areas: the ultrastructure of axons, the mechanisms of axonal degeneration in ALS, the impact of impaired axonal transport on disease progression in ALS, and the potential for axonal regeneration within the central nervous system (CNS). Our objective is to achieve a more holistic and profound understanding of the multifaceted role that axons play in ALS, thereby offering a more intricate and refined perspective on targeted axonal therapeutic interventions.
肌萎缩侧索硬化症(ALS),通常被称为运动神经元病,是一种神经退行性疾病,其特征是上下运动神经元进行性退化。这种病理过程会导致肌肉无力,并最终可能导致瘫痪。迄今为止,ALS的确切病因仍不清楚。然而,越来越多的研究表明,轴突功能障碍是ALS发病机制中的关键因素,并显著影响疾病的进展。ALS中轴突功能障碍会导致神经冲动传递受阻,进而导致运动障碍、肌肉萎缩以及其他相关并发症,严重影响患者的生活质量和生存预后。在这篇综述中,我们聚焦于几个关键领域:轴突的超微结构、ALS中轴突变性的机制、轴突运输受损对ALS疾病进展的影响以及中枢神经系统(CNS)内轴突再生的潜力。我们的目标是更全面、深入地了解轴突在ALS中所起的多方面作用,从而为靶向轴突治疗干预提供更细致、精确的观点。