Mutation of NLRP3 is responsible for rare autoinflammatory diseases, collectively referred to as cryopyrin-associated periodic syndromes (CAPS; Neven et al., 2004; Ting and Davis, 2005; Jha and Ting, 2009), characterized by the hyperactivation of the inflammasome complex and increased IL-1β (Neven et al., 2004; Goldbach-Mansky et al., 2006). This evidence concerns the gene NLRP3 and cryopyrin-associated periodic syndrome.