To determine whether overexpression of mutant TDP-43 recapitulates the formation of these accumulations in our mutant TDP-43 mice, we examined brain and spinal cord sections of Mt-TAR6/6 and Mt-TAR5/6 mice and compared them to the pathology found in Wt-TAR4/4 mice as well as to ALS-FTLD patients. Here, TARDBP is linked to amyotrophic lateral sclerosis.