Inflammasome activation and assembly in Huntington's disease

Tiago de Oliveira Furlam, Isadora Gonçalves Roque, Ewelin Wasner Machado da Silva, Pedro Parenti Vianna, Priscila Aparecida Costa Valadão, Cristina Guatimosim, Antônio Lúcio Teixeira, Aline Silva de Miranda

Research output: Contribution to journalReview articlepeer-review

6 Scopus citations

Abstract

Huntington's disease (HD) is a rare neurodegenerative disease characterized by motor, cognitive, and psychiatric symptoms. Inflammasomes are multiprotein complexes capable of sensing pathogen-associated and damage-associated molecular patterns, triggering innate immune pathways. Activation of inflammasomes results in a pro-inflammatory cascade involving, among other molecules, caspases and interleukins. NLRP3 (nucleotide-binding domain, leucine-rich-repeat containing family, pyrin domain-containing 3) is the most studied inflammasome complex, and its activation results in caspase-1 mediated cleavage of the pro-interleukins IL-1β and IL-18 into their mature forms, also inducing a gasdermin D mediated form of pro-inflammatory cell death, i.e. pyroptosis. Accumulating evidence has implicated NLRP3 inflammasome complex in neurodegenerative diseases. The evidence in HD is still scant and mostly derived from pre-clinical studies. This review aims to present the available evidence on NLRP3 inflammasome activation in HD and to discuss whether targeting this innate immune system complex might be a promising therapeutic strategy to alleviate its symptoms.

Original languageEnglish (US)
Pages (from-to)134-142
Number of pages9
JournalMolecular Immunology
Volume151
DOIs
StatePublished - Nov 2022
Externally publishedYes

Keywords

  • Caspase-1
  • Huntington's disease
  • IL-18
  • IL-1β
  • Inflammasome
  • NLRP3

ASJC Scopus subject areas

  • Immunology
  • Molecular Biology

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