Progression from pre-symptomatic to early-symptomatic ALS is associated with upregulated Nlrp3 and IL-1β gene expression, increased NLRP3 and ASC protein expression and mature IL-1β release in the SOD1(G93A) mouse spinal cord (Johann et al., 2015; Cunha et al., 2018), and NLRP3 protein expression, caspase-1 cleavage and mature IL-1β secretion in microglia is significantly increased in response to LPS (Bellezza et al., 2018). This evidence concerns the gene CASP1 and amyotrophic lateral sclerosis.