Emerging Role of NLRP3 Inflammasome/Pyroptosis in Huntington's Disease.

Int J Mol Sci

Laboratory of Neuroanatomy, IRRCS Santa Lucia, Via del Fosso di Fiorano 64, 00143 Rome, Italy.

Published: July 2022

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.

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Source
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC9368941PMC
http://dx.doi.org/10.3390/ijms23158363DOI Listing

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