Amyotrophic lateral sclerosis (ALS) is an unrelenting progressive neurodegenerative disease causing progressive weakness, ultimately leading to death. Despite aggressive research, the pathways leading to neuronal death are incompletely understood. Riluzole is the only drug clinically proven to enhance survival of ALS patients, but its mechanism of action is not clearly understood. In this article, the proposed pathophysiology of ALS is reviewed including glutamate excitotoxicity, oxidative stress, mitochondrial dysfunction, autoimmune mechanisms, protein aggregation, SOD1 accumulation, and neuronal death. Based on these mechanisms, past major ALS drug studies will be reviewed as well as promising current ALS drug studies, focusing on the advancement of these studies from the bench to the patient's bedside.

译文

肌萎缩性侧索硬化症(ALS)是一种持续不断的进行性神经退行性疾病,会导致进行性无力,最终导致死亡。尽管进行了积极的研究,导致神经元死亡的途径仍未完全了解。利鲁唑是临床上唯一被证明可提高ALS患者生存率的药物,但其作用机理尚不清楚。在本文中,对ALS的拟议病理生理进行了综述,包括谷氨酸兴奋性毒性,氧化应激,线粒体功能障碍,自身免疫机制,蛋白质聚集,SOD1积累和神经元死亡。基于这些机制,将回顾过去的主要ALS药物研究以及有前途的当前ALS药物研究,重点是这些研究从实验台到患者床边的进展。

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