In 2006, two seminal papers by Neumann et al. [1] and Arai et al. [2] reported that the nuclear TAR DNA-binding protein 43 (TDP-43) is involved in amyotrophic lateral sclerosis (ALS) and tau-negative, ubiquitin-positive frontotemporal lobar degeneration (FTLD-U). This evidence concerns the gene TARDBP and frontotemporal dementia.