NLRP3 and amyotrophic lateral sclerosis: In fact, activation of NLRP3 inflammasome complex has been previously reported in both microglia obtained from mouse models of ALS as well as ALS patients’ brain tissue samples, and TDP43 has been shown to be activate microglial NLRP3 (Johann et al., 2015; Zhao et al., 2015; Kadhim et al., 2016).