Among different types of the inflammasomes, the NLRP3 (NLR family pyrin domain containing 3) inflammasome is the most well-characterized inflammasome (Hise et al. 2009) and contributes to the development of a number of monogenic autoinflammatory diseases, including the inherited CAPSs Muckle–Wells syndrome (MWS), familial cold autoinflammatory syndrome and neonatal-onset multisystem inflammatory disease (Masters et al. 2009), as well as various metabolic and neurodegenerative disorders(Wen et al. 2012), (De et al. 2014). Here, NLRP3 is linked to CINCA syndrome.