In this context, a chromogranin-mediated secretion of mutant SOD1, but not wild-type SOD1 proteins, linked to ALS progression, has been observed [16], while a clear association of microglia activation with the occurrence of motor neurons toxicity has been revealed in transgenic mice carrying SOD1 mutations [16,99]. The gene discussed is SOD1; the disease is amyotrophic lateral sclerosis.