TDP-43 was first associated with neurodegenerative diseases in 2006 by Neumann et al. and Arai et al. [11], when the protein was identified as a major protein component in insoluble inclusions of patients with Frontotemporal Lobal Degeneration with Ubiquitin-positive inclusions (FTLD-U) and Amyotrophic Lateral Sclerosis (ALS) patients [12]. Here, TARDBP is linked to amyotrophic lateral sclerosis.