TARDBP and early-onset autosomal dominant Alzheimer disease: Although only a small fraction of familial cases are caused by mutations in trans-active response element DNA binding protein (TDP-43), pathological misfolding and cytoplasmic aggregation of this protein is seen in 97% of ALS patients, 45% of FTD patients, and 57% of Alzheimer’s Disease patients (AD) [3–5].