To date, hundreds of mutations in more than 20 genes have been implicated in the pathology of ALS, whereby mutations in genes coding for superoxide dismutase 1 (SOD1), TAR DNA-binding protein 43 (TDP-43) and fused in sarcoma (FUS) as well as a hexanucleotide expansion on chromosome 9 in open reading frame 72 (C9ORF72) account for the most cases with a familial background [1-6]. The gene discussed is FUS; the disease is amyotrophic lateral sclerosis.