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

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.