To model ALS, we used transgenic mice expressing SOD1 with glycine 93 mutated to alanine (SOD1-G93A) (Fig. 3a, top panel) [29], and to model SBMA we used transgenic mice expressing AR with a polyQ tract of 100 glutamine residues (AR100Q) (Fig. 3b, top panel) [26, 30]. This evidence concerns the gene SOD1 and amyotrophic lateral sclerosis.