Inflammasomes and related diseases

Abstract

  Although inflammation is important for host defense, excessive inflammation sometimes causes serious consequences. IL-1β is one of major proinflammatory cytokines. Dysregulation of IL-1β promotes development of several diseases. Mature IL-1β is produced by cleavage of its proform by a protein complex named inflammasome. Inflammasome consists of NOD-LRRs containing family (NLR proteins), an adaptor protein, and a cysteine protease caspase-1. Several NLRs can be assembled into inflammasome in response to various stimulatory signals.
  Genetic disorder of inflammasome-IL-1 system cause autoinflammatory diseases such as cryopyrin-associated autoinflammatory disease, familial Mediterranean fever, deficiency of IL-1 receptor antagonist, and PAPA syndrome. This article reviews recent advances in the study of inflammasome and related diseases.