Severe overexpression of TDP-43ΔNLS causes neurodegeneration and molecular signatures associated with ALS (Igaz et al., 2011), mimicking the cytoplasmic mis-localization of TDP-43 identified as a pathological hallmark of motor neurons in 97% of all ALS cases (Mackenzie et al., 2007; Maekawa et al., 2009). This evidence concerns the gene TARDBP and amyotrophic lateral sclerosis.