As with TDP-43, FUS is mislocalized from the nucleus and found aggregated in the cytoplasm in ALS post-mortem tissues (FUS proteinopathy) and mammalian cell models (Mackenzie et al., 2011; Shang and Huang, 2016; Sharma et al., 2016). The gene discussed is TARDBP; the disease is amyotrophic lateral sclerosis.