These specific forms of TDP-43 are present in patients with ALS and FTD [31] and in the cerebellum and hippocampus of rats treated with intrathecal infusion of BMAA, as well as in the spinal cord where mild accumulation and aggregation of TDP-43 in the cytosol of some injured and degenerating motor neurons were observed [26]. Here, TARDBP is linked to frontotemporal dementia.