While accumulating evidence underscores a critical connection between genome damage and neuron loss in ALS-TDP-43 and related diseases, to date, only rNLS8 (hTDP-43∆NLS transgenic) line has shown approximately 2–3 folds overexpression of DNA damage-inducible transcript 3 (Chop), and growth arrest and DNA-damage-inducible 45 gamma (Gadd45γ), as the early markers of cellular stress and death [74–76]. This evidence concerns the gene TARDBP and amyotrophic lateral sclerosis.