The aggregation of TDP-43 has been largely investigated since it mostly occurs in absence of mutations within the associated TARDBP gene (which count for <10% of the familial ALS cases; Kabashi et al., 2008; Sreedharan et al., 2008) and has been also observed in different neurological disorders such as frontotemporal dementia and Alzheimer's disease (Arai et al., 2006; Amador-Ortiz et al., 2007). This evidence concerns the gene TARDBP and early-onset autosomal dominant Alzheimer disease.