Protein aggregates containing phosphorylated proteins is a key pathological feature in many neurodegenerative diseases, such as phospho-tau in Alzheimer’s disease and FTD (Sjogren et al., 2001), phospho-TDP-43 in ALS and FTD (Hasegawa et al., 2008) and phospho-alpha-synuclein in Parkinson’s disease (Wang et al., 2012). The gene discussed is MAPT; the disease is early-onset autosomal dominant Alzheimer disease.