More than 50 different point mutations in the TARDBP gene (encoding for TDP-43) have been identified as ALS-causing (frequency: 4.2% in fALS; 0.8% in sALS) [34,35], altering its shuttle role from the nucleus to the cytoplasm and therefore modulating its RNA-related functions and determining the formation of aggregates [47]. This evidence concerns the gene TARDBP and amyotrophic lateral sclerosis.