Although aberrant APA events caused by TDP-43 depletion have been systematically analyzed and validated in recent studies, the extent to which these APA changes are reflected in the brain of individuals with frontotemporal lobar degeneration with TDP-43 pathology (FTLD-TDP) remain to be elucidated, as do the functional outcomes of these events. Here, TARDBP is linked to frontotemporal dementia.