Besides FTLD and ALS, some degree of neuronal TDP-43 pathology has also been reported in a variety of additional neurodegenerative diseases, including Alzheimer’s disease (up to 60% of the patients) [1, 36], corticobasal degeneration (CBD) [72], progressive supranuclear palsy (PSP) [80], Parkinson’s disease [18] and Huntington’s disease [23, 62]. The gene discussed is TARDBP; the disease is amyotrophic lateral sclerosis.