TDP-43 pathology in the absence of TARDBP mutations is also found in hippocampal sclerosis of the elderly, as well as a subset of patients with Alzheimer’s disease, Parkinson’s disease or other neurodegenerative disorders [2, 3, 15, 17, 18, 29, 37], suggesting a pervasive involvement of TDP-43 in neurodegeneration. This evidence concerns the gene TARDBP and early-onset autosomal dominant Alzheimer disease.