While accumulating evidence underscores a critical connection between genome damage and neuron loss in ALS/FTD-TDP-43 and related diseases, to date, only rNLS8 (hTDP-43ΔNLS transgenic) line has shown approximately 2-3 folds overexpression of DNA-damage inducible transcript 3 (Chop), growth arrest, and DNA-damage-inducible 45 gamma (Gadd45γ), as the early markers of cellular stress and death [67-69]. This evidence concerns the gene TARDBP and frontotemporal dementia.