Humans harboring one of many NLRP3 point mutations8 suffer from diseases that result in Familial cold-induced autoinflammatory syndrome (FCAS), Muckle-Wells Syndrome (MWS), Chronic infantile neurologic cutaneous articular (CINCA) syndrome/Neonatal-onset multisystem inflammatory disease (NOMID), which are collectively called cryopyrin-associated periodic syndromes (CAPS) due to constitutively active NLRP39, are more sensitive to activating stimuli. Here, NLRP3 is linked to cryopyrin-associated periodic syndrome.