FUS and frontotemporal dementia: To examine the underlying mechanism of FUS proteinopathy during the early stage of ALS and FTD, we first compared the transcriptome data generated from 6-month-old wild-type (Wt) and ΔNLS-FUS Tg mice, in which exogenously overexpressed mutant FUS (ΔNLS-FUS) was mislocalized in the cytoplasm (see Supplementary Fig. S1) and significant motor deficits appear9.