In order to study the effects of mutant TDP-43, we have generated transgenic zebrafish lines expressing stably either human wild-type TDP-43 (TDP-43WT) or bearing the G348C mutations found in ALS patients from a French-Canadian population (TDP-43G348C) (Kabashi et al., 2008). Here, TARDBP is linked to amyotrophic lateral sclerosis.