Patients with FTD due to GRN mutations (FTD-GRN) develop frontotemporal lobar degeneration with TAR DNA-binding protein 43 (TDP-43) pathology type A, which is characterized by TDP-43 aggregation and neuronal loss, particularly in layer II/III of the cortex [3]. The gene discussed is TARDBP; the disease is frontotemporal dementia.