Given that multiple (although not all) forms of both sporadic and familial FTD/ALS cases of different genetic origin (i.e., mutations in C9orf72, progranulin, TARDBP, etc.)converge in a common pathological presentation that involves TDP-43 dysregulation (Seelaar et al., 2011; Renton et al., 2014), there is a growing need in the field for understanding the pathological and behavioral consequences of these abnormalities. Here, TARDBP is linked to amyotrophic lateral sclerosis.