Several recent studies suggest the NLRP3 inflammasome is also activated by TDP-43, an RNA-binding protein which forms pathological intraneuronal inclusions in the majority of cases of FTD/ALS.56 Upregulation of NLRP3, ASC and caspase-1 mRNA is observed in the spinal cord of mice expressing an ALS-linked TDP-43 mutation, and treatment with WT or mutant TDP-43 peptide causes IL-1β release in mouse primary microglia. This evidence concerns the gene NLRP3 and amyotrophic lateral sclerosis.