Figure 1.

The NLRP3 inflammasome is generally assembled upon activation by a range of pathogen-associated molecular patterns through pattern recognition receptors (PRRs) such as TLRs (eg, LPS activation of TLR4), or by interaction with components of internalized damage associated molecular pattern molecules (eg, uric acid crystals) leading to IL-1 cytokine maturation. Mutations to the NACHT domain of the NLRP3 subunit can result in spontaneous activation of the inflammasome and may lead to over-production and secretion of IL-1β, which is thought to be a causative factor in the clinical manifestations of CAPS.