TDP-43 pathology is a hallmark of neurodegeneration and is present in ~97 % of ALS cases, ~45 % of cases with frontotemporal dementia (FTD) (Ling et al., 2013), and ~60 % of AD cases (Amador-Ortiz et al., 2007; Arai et al., 2009; Uryu et al., 2008). This evidence concerns the gene TARDBP and frontotemporal dementia.