This 43 kDa protein is encoded by the TARDBP gene on chromosome 1, and TARDBP mutations have been genetically linked to ubiquitin-positive, Tau-negative inclusions in Amyotrophic Lateral Sclerosis (ALS) and subtypes of Frontotemporal Lobar Degeneration (FTLD) linked to TDP-43 (FTLD-TDP) and motor neuron disease (FTLD-MND) [2]. This evidence concerns the gene TARDBP and frontotemporal dementia.