Using two mouse models displaying GOF mutations in NLRP3 (Nlrp3A350V and Nlrp3L351P) associated with two clinical entities of CAPS, namely Muckle-Wells syndrome and familial cold autoinflammatory syndrome [135], authors demonstrated that targeted and specific GOF mutations in NLRP3 in neutrophils (Nlrp3A350VMRP8cre and Nlrp3L351PMRP8cre mice model) were sufficient to trigger severe CAPS-like phenotype as judged by the intense extent of skin inflammation [98]. This evidence concerns the gene NLRP3 and Muckle-Wells syndrome.