Aggregated and soluble SOD1-G93A activate the NLRP3 inflammasome in primary mouse microglia, leading to IL-1β release, whereas NLRP3-deficient microglia do not show this response, indicating a specific role for NLRP3 in ALS neurotoxicity (Deora et al., 2020). Here, NLRP3 is linked to amyotrophic lateral sclerosis.