Johann et al. [127] showed that an activated NLRP3 inflammasome concurred with elevated levels of caspase-1, IL-1β, and IL-18, particularly in the spinal cord astrocytes of the SOD1G93A ALS-model mice and in the serum and spinal cord tissue of sporadic ALS patients—altogether findings confirming NLRP3 inflammasome’s involvement in ALS. This evidence concerns the gene NLRP3 and amyotrophic lateral sclerosis.