The inflammasomes and autoinflammatory syndromes

Lori Broderick, Dominic De Nardo, Bernardo S. Franklin, Hal M. Hoffman, Eicke Latz

Research output: Contribution to journalReview ArticleResearchpeer-review

174 Citations (Scopus)

Abstract

inflammation, a vital response of the immune system to infection and damage to tissues, can be initiated by various germline-encoded innate immune-signaling receptors. Among these, the inflammasomes are critical for activation of the potent proinflammatory interleukin-1 cytokine family. Additionally, inflammasomes can trigger and maintain inflammatory responses aimed toward excess nutrients and the numerous danger signals that appear in a variety of chronic inflammatory diseases. We discuss our understanding of how inflammasomes assemble to trigger caspase-1 activation and subsequent cytokine release, describe how genetic mutations in inflammasome-related genes lead to autoinflammatory syndromes, and review the contribution of inflammasome activation to various pathologies arising from metabolic dysfunction. Insights into the mechanisms that govern inflammasome activation will help in the development of novel therapeutic strategies, not only for managing genetic diseases associated with overactive inflammasomes, but also for treating common metabolic diseases for which effective therapies are currently lacking.

Original languageEnglish
Pages (from-to)395-424
Number of pages30
JournalAnnual Reviews of Pathology: Mechanisms of Disease
Volume10
DOIs
Publication statusPublished - 1 Jan 2015
Externally publishedYes

Keywords

  • AIM2
  • ASC
  • CAPS
  • FMF
  • inflammasomes
  • NLRC4
  • NLRP1
  • NLRP12
  • NLRP3
  • NLRP6

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