Given that TDP-43 pathology is closely tied to neurodegeneration in FTD and ALS (Mackenzie et al., 2013, 2015), the model represents one of the few animal models that trigger aggregation of endogenous WT TDP-43 and will be useful for studying the mechanisms of endogenous TDP-43 aggregation. The gene discussed is TARDBP; the disease is frontotemporal dementia.