NLRP2, also known as NALP2 or PAN1, is a member of the pyrin domain-containing NLR protein (NLRP) family. The NLR proteins interact with ASC via the conserved pyrin domain, which results in the activation of caspase-1 and maturation of the pro-inflammatory cytokines IL-1β and IL-18. NLRP2 is also involved in the formation of the inflammasome and caspase-1 activation. NLRP2 negatively regulates NFκB activation, which is induced by TNF-α, through inhibiting NFKBIA degradation. In addition, NLRP2 is one of the maternal effect genes and plays a crucial role in embryonic development. In mouse embryos, knockdown or mutation of NLRP2 leads to developmental defections. Truncation of the NLRP2 gene is reported to associate with an overgrowth discorder called Beckwith-Wiedemann Syndrome.