In 2006 it was reported, for the first time, that TDP-43 is the main component of the ubiquitin-positive, tau-negative and α-synuclein-negative protein inclusions accumulating in the fronto-temporal cortex and hippocampus of the brain and in the motor neurons of the spinal cord of patients suffering from frontotemporal lobar degeneration with ubiquitin-positive inclusions (FTLD-U) and amyotrophic lateral sclerosis (ALS) [4]. Here, TARDBP is linked to frontotemporal dementia.