This gene encodes a pyrin-like protein containing a pyrin domain， a nucleotide-binding site (NBS) domain， and a leucine-rich repeat (LRR) motif. This protein interacts with the apoptosis-associated speck-like protein PYCARD/ASC， which contains a caspase recruitment domain， and is a member of the NLRP3 inflammasome complex. This complex functions as an upstream activator of NF-kappaB signaling， and it plays a role in the regulation of inflammation， the immune response， and apoptosis. The SARS-CoV 3a protein， a transmembrane pore-forming viroporin， has been shown to activate the NLRP3 inflammasome via the formation of ion channels in macrophages. Mutations in this gene are associated with familial cold autoinflammatory syndrome (FCAS)， Muckle-Wells syndrome (MWS)， chronic infantile neurological cutaneous and articular (CINCA) syndrome， neonatal-onset multisystem inflammatory disease (NOMID)， keratoendotheliitis fugax hereditarian， and deafness， autosomal dominant 34， with or with