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.
Meng
Guangxun
G
Mucosal Immunity Section, Laboratory of Host Defense, NIAID, NIH Bethesda, MD 20892, USA.
Strober
Warren
W
D000818:Animals; D001327:Autoimmune Diseases / Q000235:genetics* / Q000276:immunology; D002352:Carrier Proteins / Q000235:genetics* / Q000276:immunology; D007249:Inflammation / Q000235:genetics* / Q000276:immunology; D051379:Mice; D009154:Mutation; D000071199:NLR Family, Pyrin Domain-Containing 3 Protein
D002352:Carrier Proteins; D000071199:NLR Family, Pyrin Domain-Containing 3 Protein; C453881:NLRP3 protein, human