Firstly, levels of NLRP3 and its adaptor molecule apoptosis-associated speck-like protein (ASC) as well as active caspase-1, IL-1β and IL-18 are elevated in the spinal cord, motor cortex and/or skeletal muscle of ALS patients and in the mutant superoxide dismutase 1 (SOD1) mouse model of ALS (17–21). This evidence concerns the gene IL1B and amyotrophic lateral sclerosis.