SOD1 and amyotrophic lateral sclerosis: Taken together, these results suggest the two faced action of the NLRP3 inflammasome in ALS: where, at the early stage of the disease, it plays a beneficial role by clearing noxious aggregates, while, when the disease progresses, the chronic NLRP3 stimulations by an excess of damage signals, such as mutant proteins SOD1 and TDP-43, change its positive effect into a harmful action leading to myofiber damage and, ultimately, to motoneuron degeneration [169,170].