Neuropathologically, abnormal subcellular localization and aggregation of TAR DNA binding protein 43 (TDP-43) is found widely distributed in most patients with ALS and FTD, cortical lesions are TDP-43 positive but cerebellar lesions are negative (Al-Sarraj et al., 2011; Ince et al., 2011; Stewart et al., 2012). Here, TARDBP is linked to frontotemporal dementia.