Although AD is frequently pathologically characterized by the accumulation of extracellular Aβ plaques (Hardy and Higgins, 1992), other proteinopathies are often present including neurofibrillary tangles of intracellular hyperphosphorylated tau protein, aggregates of TAR DNA-binding protein 43 (TDP-43), and α-synuclein pathology as seen in other causes of dementia (Josephs et al., 2014; DeTure and Dickson, 2019). Here, TARDBP is linked to Alzheimer disease.