Indeed, the activation of the NLRP3 inflammasome has been observed, in brain, spinal cord and in the skeletal muscle of SOD1G93A mice, a transgenic mouse model of ALS expressing a mutant form of human Superoxide Dismutase 1 (SOD1), [158,159], as well as in those of sporadic ALS (sALS) patients [160,161,162]. This evidence concerns the gene NLRP3 and amyotrophic lateral sclerosis.