The neuropathological hallmark of the associated FTLD includes foci of dinucleotide-peptide repeats (DPRs) in specific neuronal subpopulations in association with more widespread pathological TDP-43 deposition [reviewed in (36, 91)] in the absence of an associated tauopathy or when present, as a non-specific finding (92). This evidence concerns the gene TARDBP and tauopathy.