However, direct evidence linking excessive NLRC4 inflammasome activation to human disease comes from two pioneering reports in 2014, which identified gain-of-function mutations in NLRC4 (specifically T337S and V341 A) as contributors to the pathogenesis of autoinflammatory enterocolitis (AIFEC) [10, 11]. Here, NLRC4 is linked to periodic fever-infantile enterocolitis-autoinflammatory syndrome.