Neuropathological classification of Frontotemporal Dementia (FTD) is based on the major constituents of the cellular inclusions present, such as tau, TAR-DNA-binding protein-43 (TDP-43) or fused-in-sarcoma (FUS) protein, designated FTLD-tau, FTLD-TDP or FTLD-FUS respectively [8]. Here, TARDBP is linked to frontotemporal dementia.