Since NLRP12 has a close structural homology with NLRP3, we investigated whether, as in patients with CAPS, IL-1β hypersecretion in response to Toll-like receptor (TLR) stimulation may also represent the crucial pathogenic event in patients carrying the p.Asp294Glu NLRP12 mutation. Here, IL1B is linked to cryopyrin-associated periodic syndrome.