The upregulation of NLRP3 inflammasome was widely demonstrated in the microglia of C9orf72- [176], hSOD1-, and hTDP-43-related ALS mouse models [177,178,179], and increased amounts of NLRP3, ASC, and caspase 1 were found both in postmortem tissue and in the serum of ALS patients [180,181]. This evidence concerns the gene NLRP3 and amyotrophic lateral sclerosis.