Department of Neurosciences, Experimental Neurology, and Leuven Brain Institute (LBI), KU Leuven-University of Leuven, 3000, Leuven, Belgium.
Laboratory of Neurobiology, Experimental Neurology, Center for Brain and Disease Research, VIB, Campus Gasthuisberg, O&N5, Herestraat 49, 602, 3000, Leuven, PB, Belgium.
Mol Neurodegener. 2022 Mar 18;17(1):22. doi: 10.1186/s13024-022-00525-z.
Neuroinflammation is an important hallmark of amyotrophic lateral sclerosis (ALS) and frontotemporal lobar degeneration (FTLD). An inflammatory reaction to neuronal injury is deemed vital for neuronal health and homeostasis. However, a continued activation of the inflammatory response can be detrimental to remaining neurons and aggravate the disease process. Apart from a disease modifying role, some evidence suggests that neuroinflammation may also contribute to the upstream cause of the disease. In this review, we will first focus on the role of neuroinflammation in the pathogenesis of chromosome 9 open reading frame 72 gene (C9orf72) hexanucleotide repeat expansions (HRE)-mediated ALS/FTD (C9-ALS/FTD). Additionally, we will discuss evidence from ex vivo and in vivo studies and finally, we briefly summarize the trials and progress of anti-inflammatory therapies.
神经炎症是肌萎缩侧索硬化症（ALS）和额颞叶变性（FTLD）的重要标志。神经元损伤引起的炎症反应被认为对神经元的健康和内稳态至关重要。然而，炎症反应的持续激活可能对剩余神经元有害，并加重疾病进程。除了疾病修饰作用外，一些证据表明神经炎症也可能导致疾病的上游原因。在这篇综述中，我们将首先关注神经炎症在染色体 9 开放阅读框 72 基因（C9orf72）六核苷酸重复扩增（HRE）介导的 ALS/FTD（C9-ALS/FTD）发病机制中的作用。此外，我们将讨论来自体外和体内研究的证据，最后，我们简要总结抗炎治疗的试验和进展。
