The emergence of predominantly cognitive FTD-like phenotypes in the TDP-43K408R mice is not unusual in TDP-43 knock in models such as TDP-43Q331K and TDP-43K145R mice which present with features of cognitive dysfunction but lacking the entire constellation of ALS/FTD-like phenotypes [45, 113]. This evidence concerns the gene TARDBP and amyotrophic lateral sclerosis.