Since 2009, several mutations in the FUS gene have been linked to ALS (with a frequency of 2.8% for fALS cases and 0.3% for sALS cases) [30,31,32,33,34,35], many of which affect FUS nuclear localization, impairing its role as a regulator of transcription and RNA maturation and causing toxic FUS aggregates in the cytoplasm [36,37,38,39,40,41]. Here, FUS is linked to amyotrophic lateral sclerosis.