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The identification of TDP43 as the major protein in the neuropathological hallmark lesions of patients with frontotemporal lobar degeneration with ubiquitinated inclusions (FTLDU, meanwhile renamed to FTLDTDP (Mackenzie et al, 2009)) and in amyotrophic lateral sclerosis (ALS) emphasizes the importance of TDP43 (Neumann et al, 2006). Disti