Fig. 8

NLRC4 inflammasomopathies are related to gain-of-function mutations of NLRC4 inflammasome. Gain-of-function of mutations in NLRC4 in patients with NLRC4 inflammasomopathies constitutively actives for NLRC4 inflammasome. The NLRC4 inflammasome activates caspase-1 either with or without an adaptor ASC leading to hypersecretion of IL-18 and IL-1β