Mutations in the<unk>  gene have been associated with a spectrum of dominantly inherited autoinflammatory diseases called cryopyrin-associated periodic syndrome (CAPS). This includes familial cold autoinflammatory syndrome (FCAS), Muckle–Wells syndrome (MWS), chronic infantile neurological cutaneous and articular (CINCA) syndrome, neonatal onset multisystem inflammatory disease (NOMID), and keratoendotheliitis fugax hereditaria. Defects in this gene have also been linked to familial Mediterranean fever. In addition, the<unk>  inflammasome has a role in the pathogenesis of gout, hemorrhagic stroke and neuroinflammation occurring in protein-misfolding diseases, such as Alzheimer's, Parkinson's, and prion diseases. Amelioration of mouse models of many diseases has been shown to occur by deletion of the<unk>  inflammasome, including gout, type 2 diabetes, multiple sclerosis, Alzheimer's disease, and atherosclerosis. The compound β-Hydroxybutyrate has been shown to block<unk>  activation, and thus may be of benefit for many of these diseases. Deregulation of<unk>  has been connected with carcinogenesis. For example, all the components of the<unk>  inflammasome are downregulated or completely lost in human hepatocellular carcinoma.
NLRP3
Caspase-9
PTPRC
Rho
MFAP4
IKK2
ILC2
Fc receptor