New insights into the nature of autoinflammatory diseases from mice with Nlrp3 mutations

Abstract Mutations in the Nlrp3 ( CIAS1 , cryopyrin) gene are associated with cryopyrin‐associated periodic syndrome, autoinflammatory diseases characterized by excessive IL‐1 production and neutrophilia in blood and tissues. Recent studies with gene‐targeted mice expressing mutations homologous to those found in cryopyrin‐associated periodic syndrome patients have advanced the understanding of NLRP3‐associated autoinflammation. In this Viewpoint , we will discuss the mechanisms of NLRP3 inflammasome activation and its induction of Th17‐cell‐dominant immunologic responses.