Preclinical models have primarily focussed on the use of mutations in Cu/Zn superoxide dismutase (SOD)1, transactive response DNA binding protein 43 kDa (TDP-43) and fused in sarcoma/translated in liposarcoma (FUS), as well as C9ORF72 repeats, which, although infrequent, are the most common ALS susceptibility genes [1]. Here, TARDBP is linked to amyotrophic lateral sclerosis.