Over-expression of full-length TDP-43 in a variety of transgenic animal models lead to the presence of phosphorylated TDP-43 aggregates similar to ALS and FTD cases (Wegorzewska et al., 2009; Shan et al., 2010; Stallings et al., 2010; Wils et al., 2010; Xu et al., 2010a). This evidence concerns the gene TARDBP and frontotemporal dementia.