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

Int J Mol Sci. 2022 Jul 28;23(15):8363. doi: 10.3390/ijms23158363.

Abstract

Huntington's disease (HD) is a neurodegenerative disease characterized by several symptoms encompassing movement, cognition, and behavior. The mutation of the IT15 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.

Keywords: Huntington’s disease; NLRP3 inflammasome; neuroinflammation.

Publication types

  • Review

MeSH terms

  • Humans
  • Huntingtin Protein / genetics
  • Huntington Disease* / metabolism
  • Inflammasomes
  • Inflammation
  • NLR Family, Pyrin Domain-Containing 3 Protein / genetics
  • Neurodegenerative Diseases*
  • Pyroptosis

Substances

  • Huntingtin Protein
  • Inflammasomes
  • NLR Family, Pyrin Domain-Containing 3 Protein

Grants and funding

This research received no external funding.