Main diseases related to TDP-43 pathology comprise most sporadic forms of ALS and about 50% of FTD (FTD-TDP), especially behavioral variant and semantic primary progressive aphasia [23, 24] as well as cognitive impairment associated with limbic-predominant age-related TDP-43 encephalopathy [25] and Perry syndrome [26]. Here, TARDBP is linked to amyotrophic lateral sclerosis.