This was followed in 2009 by the identification of mutations in fused in sarcoma (FUS) in ∼5% of familial ALS (ALS-FUS) (Kwiatkowski et al., 2009; Vance et al., 2009) and the subsequent recognition of FUS aggregation together with TAF15 and EWS as hallmark lesions in ∼10% of sporadic FTLD cases (FTLD—FUS) (Munoz et al., 2009; Neumann et al., 2009b,c). The gene discussed is EWSR1; the disease is amyotrophic lateral sclerosis.