Mutations in the gene for microtubule-associated protein tau (MAPT) on chromosome 17 is responsible for 10–20% of familial cases and results in tau-positive inclusions pathology, while mutations in the progranulin (PGRN) gene, also on chromosome 17, is the cause for many other cases, resulting in frontotemporal dementia with the presence of ubiquitin positive inclusions containing TDP-43, accounting for 5–10% of cases [3, 14–17]. Here, GRN is linked to frontotemporal dementia.