Primary tauopathies, in which abnormal-modified tau intracellular inclusions are found in the autopsy of patients, include major forms of Frontotemporal Lobar Degeneration (FTLD) neuropathology, in which the MAPT gene located in chromosome 17 is affected (with more than 40 different pathogenic mutations) [275]; Progressive Supranuclear Palsy (PSP which is largely sporadic. Here, MAPT is linked to frontotemporal dementia.