Pathological TDP-43 is also evident in approximately 30–70% of Alzheimer’s disease patients, particularly those with fast progressing disease and advanced β-amyloid plaque and neurofibrillary tangle pathology (Josephs et al., 2016), and the presence of TDP-43 pathology is associated with mutation to the APOE ε4 gene independent of β-amyloid pathology (Wennberg et al., 2018). The gene discussed is TARDBP; the disease is early-onset autosomal dominant Alzheimer disease.