Whereas nuclear loss and cytoplasmic accumulation of TDP-43 are the most prominent features of the pathology of ALS and frontotemporal dementia (Arai et al, 2006; Neumann et al, 2006), mutations in the COOH-terminal region of FUS result in its mislocalization to the cytoplasm and nuclear depletion, both of which are considered to be central to ALS pathogenesis (Akiyama et al, 2019; Kwiatkowski et al, 2009; Suzuki et al, 2010; Suzuki et al, 2012). The gene discussed is FUS; the disease is amyotrophic lateral sclerosis.