To explore the molecular mechanism underlying FUS proteinopathy in FTD, we examined tg mice overexpressing Myc-tagged exogenous ΔNLS-FUS under Thy-1 promoter, which developed progressive motor weakness from 20 weeks of age and formed ubiquitin/p62-positive cytoplasmic aggregates from 6 months of age (Shiihashi et al., 2016). Here, FUS is linked to frontotemporal dementia.