Various studies have demonstrated that the NLRP3 inflammasome triggers a pathogenic inflammatory response in many inherited myopathies, including limb girdle muscular dystrophy type 2B (LGMD2B) [30], valosin-containing protein (VCP) associated diseases [79], and Duchene muscular dystrophy (DMD) [80,135]. Here, NLRP3 is linked to autosomal recessive limb-girdle muscular dystrophy type 2B.