In 2006, almost simultaneously, two research groups reported TDP-43 to be a major ubiquitinated protein component of insoluble cytoplasmic aggregates in degenerating neurons found in patients with frontotemporal dementia (FTD) and a fatal motor neuron disease, amyotrophic lateral sclerosis (ALS) [35,36]. This evidence concerns the gene TARDBP and frontotemporal dementia.