The inflammasome is a multiprotein complex that mediates the activation of caspase-1, which promotes, amongst others, the secretion of the proinflammatory cytokines interleukin (IL)-1β and IL-18. Members of the Nod-like receptor family, including NLRP3/NALP3, are critical components of the inflammasome that link danger-signals to caspase-1 activation. Defects in NLRP3 are the cause of familial cold autoinflammatory syndrome type 1 (FCAS1), Muckle-Wells syndrome (MWS) and of chronic infantile neurologic cutaneous and articular syndrome (CINCA).