Six different ALS-linked TDP-43 mutants were examined: A315T and M337V, which have been reported in multiple familial ALS pedigrees [38], [39], [40], [41], [42], [43]; D169G, the only ALS-linked mutation identified that lies outside the C-terminal region [39]; and G294A, Q331K and N390D, which have been identified in sporadic ALS patients [39], [40]. The gene discussed is TARDBP; the disease is amyotrophic lateral sclerosis.