The TDP-43 rNLS8 model offers significant improvements over previous TDP-43 in vivo models in replicating the combined core features of ALS/FTD pathology: motor neuron loss, muscle denervation/atrophy, and a progressive motor phenotype with premature death (Walker et al., 2015). The gene discussed is TARDBP; the disease is amyotrophic lateral sclerosis.