Laboratory of Neuroanatomy, IRRCS Santa Lucia, Via del Fosso di Fiorano 64, 00143 Rome, Italy.
Department of Systems Medicine, University of Rome Tor Vergata, 00143 Rome, Italy.
Int J Mol Sci. 2022 Jul 28;23(15):8363. doi: 10.3390/ijms23158363.
Huntington's disease (HD) is a neurodegenerative disease characterized by several symptoms encompassing movement, cognition, and behavior. The mutation of the gene encoding for the huntingtin protein is the cause of HD. Mutant huntingtin interacts with and impairs the function of several transcription factors involved in neuronal survival. Although many mechanisms determining neuronal death have been described over the years, the significant role of inflammation has gained momentum in the last decade. Drugs targeting the elements that orchestrate inflammation have been considered powerful tools to treat HD. In this review, we will describe the data supporting inflammasome and NLRP3 as a target of therapeutics to fight HD, deepening the possible mechanisms of action underlying these effects.
亨廷顿病(HD)是一种神经退行性疾病,其特征是存在多种运动、认知和行为症状。编码亨廷顿蛋白的基因突变是 HD 的病因。突变的亨廷顿蛋白与几种参与神经元存活的转录因子相互作用并损害其功能。尽管多年来已经描述了许多决定神经元死亡的机制,但炎症在过去十年中发挥了重要作用。靶向炎症调控因子的药物已被认为是治疗 HD 的有效工具。在这篇综述中,我们将描述支持炎症小体和 NLRP3 作为治疗 HD 的靶点的相关数据,深入探讨这些作用的潜在作用机制。