Department Neurology, Shanxi Provincial Peoples Hospital: Fifth Hospital of Shanxi Medical University, Taiyuan, 030012, China.
Beijing Ai-Si-Kang Medical Technology Co. Ltd., No. 18 11th St Economical & Technological Development Zone, Beijing, 100176, China.
J Neurol. 2024 Aug;271(8):4826-4851. doi: 10.1007/s00415-024-12485-z. Epub 2024 Jun 13.
Amyotrophic lateral sclerosis (ALS) is a fatal disease. As its pathological mechanisms are not well understood, there are no efficient therapeutics for it at present. While it is highly heterogenous both etiologically and clinically, it has a common salient hallmark, i.e., aberrant protein aggregation (APA). The upstream pathogenesis and the downstream effects of APA in ALS are sophisticated and the investigation of this pathology would be of consequence for understanding ALS. In this paper, the pathomechanism of APA in ALS and the candidate treatment strategies for it are discussed.
肌萎缩侧索硬化症(ALS)是一种致命疾病。由于其病理机制尚不清楚,目前尚无有效的治疗方法。尽管它在病因和临床方面具有高度异质性,但有一个共同的显著特征,即异常蛋白聚集(APA)。在 ALS 中,APA 的上游发病机制和下游影响复杂,对该病理学的研究对于理解 ALS 具有重要意义。本文讨论了 ALS 中 APA 的发病机制和潜在的治疗策略。