In 2006 it was reported, for the first time, that ubiquitin-positive, tau- and α-synuclein-negative intracellular inclusions found in the spinal cord motor neurons, in the hippocampus and neocortex of sporadic ALS and FTLD-U patients, contained the previously unidentified TDP-43 protein or its C-terminal fragments [4]. Here, TARDBP is linked to amyotrophic lateral sclerosis.