In the context of this review, it should be mentioned that FUS mutations have been found in patients affected by Amyotrophic Lateral Sclerosis (ALS), and Fronto-Temporal Lobar Degeneration (FTLD), two related yet distinct neurodegenerative disorders, and more recently in Essential Tremor (ET) [85,86,87]. Here, FUS is linked to amyotrophic lateral sclerosis.