School of Medicine and Surgery, University of Milano-Bicocca, 20900 Monza, Italy.
Biomolecules. 2023 Oct 26;13(11):1582. doi: 10.3390/biom13111582.
Amyotrophic lateral sclerosis (ALS) is a fatal condition characterized by the selective loss of motor neurons in the motor cortex, brainstem, and spinal cord. Muscle involvement, muscle atrophy, and subsequent paralysis are among the main features of this disease, which is defined as a neuromuscular disorder. ALS is a persistently progressive disease, and as motor neurons continue to degenerate, individuals with ALS experience a gradual decline in their ability to perform daily activities. Ultimately, muscle function loss may result in paralysis, presenting significant challenges in mobility, communication, and self-care. While the majority of ALS research has traditionally focused on pathogenic pathways in the central nervous system, there has been a great interest in muscle research. These studies were carried out on patients and animal models in order to better understand the molecular mechanisms involved and to develop therapies aimed at improving muscle function. This review summarizes the features of ALS and discusses the role of muscle, as well as examines recent studies in the development of treatments.
肌萎缩侧索硬化症(ALS)是一种致命的疾病,其特征是运动皮层、脑干和脊髓中的运动神经元选择性丧失。肌肉受累、肌肉萎缩以及随后的瘫痪是这种疾病的主要特征,它被定义为一种神经肌肉疾病。ALS 是一种持续进展的疾病,随着运动神经元不断退化,ALS 患者逐渐丧失进行日常活动的能力。最终,肌肉功能丧失可能导致瘫痪,给移动、沟通和自理带来重大挑战。虽然大多数 ALS 研究传统上集中在中枢神经系统的致病途径上,但人们对肌肉研究产生了浓厚的兴趣。这些研究是在患者和动物模型上进行的,目的是更好地了解所涉及的分子机制,并开发旨在改善肌肉功能的治疗方法。本综述总结了 ALS 的特征,并讨论了肌肉的作用,同时还研究了治疗方法的最新进展。