In particular, mutations in the copper/zinc superoxide-dismutase-1 gene (SOD1) [3]–[5], Tar DNA-binding protein gene (TARDBP) and, most recently discovered, the DNA/RNA-binding proteins FUS (fused in sarcoma) or TLS (translocation in liposarcoma) produce the typical adult-onset ALS phenotype, suggesting that alterations in RNA processing may play a central role in ALS pathogenesis [6]–[13]. This evidence concerns the gene SOD1 and amyotrophic lateral sclerosis.