Protein domains and exons targeted for sequencing. The upper panel in blue represents the coding sequence of the FUS gene with the sequenced exons identified with white numbers. The lower panel represents the protein with the studied functional domain. Mutations shown in black were identified in patients with amyotrophic lateral sclerosis (ALS), mutations in blue were identified in patients with frontotemporal dementia (FTD), and mutations in red were identified in patients with essential tremor (ET). *G156 and *G206S were found in FTD/ALS. **R216C was identified in ALS, ET and one control. ***M392I is associated with an increased risk of developing ET. E, Nuclear export signal; NLS, nuclear localization signal.