Mutations in the gene encoding the RNA binding protein FUS (fused in sarcoma) are causative of cases of familial ALS (Kwiatkowski et al., 2009, Vance et al., 2009) as well as instances of frontotemporal dementia (FTD) (Broustal et al., 2010, Van Langenhove et al., 2010, Yan et al., 2010). Here, FUS is linked to frontotemporal dementia.