TDP-43 proteinopathies are characterized by the dysfunction and aggregation of transactivation response element DNA-binding protein of 43 kDa (TDP-43), with ~95% of all amyotrophic lateral sclerosis (ALS) and ~50–60% of all frontotemporal lobar dementia (FTLD-TDP) cases harboring TDP-43 pathology (Neumann et al., 2006; Neumann et al., 2007; Cairns et al., 2007; Hogan et al., 2016). Here, TARDBP is linked to amyotrophic lateral sclerosis.