The presence of active caspase-1 in the spinal cord and cerebral spinal fluid of ALS patients and ALS models of mice have been recognized more than two decades ago, and in vivo studies have revealed that caspase-1 or IL-1β depletion or IL-1β inhibition resulted in prolonged survival without affecting disease onset (Iłzecka et al., 2001). The gene discussed is CASP1; the disease is amyotrophic lateral sclerosis.