Recently, the group of Pasinetti [183] showed that the genetic ablation of NLPR3-mediated innate immunity in the C9orf72 ALS mouse model significantly attenuates cerebral cortex inflammation, mortality, and neurodegeneration, and most notably mitigates behavioural impairments, suggesting that tackling NLRP3 activation may be a promising disease-modifying therapy for ALS. This evidence concerns the gene NLRP3 and amyotrophic lateral sclerosis.