The presence of tau-negative TDP-43 and ubiquitin-positive inclusion bodies is a major disease hallmark of Amyotrophic lateral sclerosis (ALS) and frontotemporal lobar degeneration (FTLD) (Arai et al., 2006; Neumann et al., 2006; Mackenzie and Rademakers, 2008; Lee et al., 2012). The gene discussed is TARDBP; the disease is frontotemporal dementia.