Although TDP-43 mutations are only associated with a small fraction of cases (Buratti, 2015), TDP-43-positive inclusions are found in the vast majority of postmortem neuronal tissue from confirmed ALS-FTLD patients (Arai et al., 2006; Neumann et al., 2009; Ling et al., 2013). The gene discussed is TARDBP; the disease is amyotrophic lateral sclerosis.