Although pathologically ubiquitinated and phosphorylated TDP-43 inclusions are commonly linked to neurodegeneration in ALS and FTLD-TDP [5,6], TDP-43 pathology has also been observed in patients with other neurodegenerative diseases such as Alzheimer’s disease (AD), Parkinson’s disease (PD), dementia with Lewy bodies (DLB), Huntington’s disease, corticobasal degeneration (CBD), progressive supranuclear palsy (PSP), Guam parkinsonism-dementia complex (G-PDC), and Perry disease [15]. This evidence concerns the gene TARDBP and Classical progressive supranuclear palsy.