Using inducible transgenic mice, we demonstrated that expression of a cytoplasmic form of TDP-43 (termed TDP-43-ΔNLS) recapitulates multiple features of TDP-43 proteinopathies of the ALS/FTD spectrum, including progressive neurodegeneration, gliosis, global gene expression changes and behavioral abnormalities encompassing the motor, cognitive and social domains (Igaz et al., 2011; Alfieri et al., 2014). The gene discussed is TARDBP; the disease is frontotemporal dementia.