Neuronal cytoplasmic inclusions of FUS are found in ALS patients with mutations in FUS (Kwiatkowski et al., 2009; Vance et al., 2009; Dormann et al., 2010), suggesting that (i) disturbing the nuclear-cytosolic distribution can lead to FUS proteinopathy, and (ii) loss of nuclear RNA processing functions may contribute to ALS pathogenesis. Here, FUS is linked to amyotrophic lateral sclerosis.