Mouse—Motor deficits and loss of cortical and cerebellar neurons similar to clinical symptoms of ALS/FLTD; RNA-foci, TDP-43 pathology; astrogliosis (Rohrer et al., 2015) Zebrafish, fly and worm—MN degeneration, more detailed studies awaiting (Therrien and Parker, 2014; Casci and Pandey, 2015). The gene discussed is TARDBP; the disease is amyotrophic lateral sclerosis.