Neurodegenerative tauopathies, including Alzheimer’s disease (AD) and frontotemporal lobar degeneration (FTLD) share a common neuropathological feature: the deposition of misfolded and hyperphosphorylated tau protein in multimeric, highly-ordered, filamentous aggregates in the central nervous system. The gene discussed is MAPT; the disease is frontotemporal dementia.