To examine a potential contribution of TDP-43 and a possible link to human disease, we used a previously described transgenic mouse line containing a mutant allele that causes the neurodegenerative disease, ALS, in human patients Prnp-TARDBP A315T (TDP43A315T) (Wegorzewska et al., 2009). The gene discussed is PRNP; the disease is amyotrophic lateral sclerosis.