We have chosen prpTDP-43A315T mice for our analyses, because this mouse model of TDP-43 pathology was generated based on the A315T mutation detected in the TARDP gene of ALS patients, who had TDP-43 pathology in their cortex19, and because the mouse model strictly recapitulated many aspects of the disease, including progressive CSMN degeneration16. The gene discussed is TARDBP; the disease is amyotrophic lateral sclerosis.