Moreover, several C9ALS/FTD mouse models reported that neurodegeneration only occurred in the presence of TDP-43 pathology (Chew et al., 2015; O’Rourke et al., 2015; Peters et al., 2015; Liu et al., 2016), and clinico-pathological evidence (Proudfoot et al., 2014; Baborie et al., 2015; Vatsavayai et al., 2016) revealed DPR pathology precedes that of TDP-43. This evidence concerns the gene TARDBP and frontotemporal dementia.