Less frequently, mutations in the genes encoding TAR DNA-binding protein 43 (TDP-43), valosin containing protein (VCP), and the charged multivesicular body protein 2B (CHMP2B), Ubiquilin 2 (UBQLN2), prion protein (PRNP) and Triggering Receptor Expressed on Myeloid Cells 2 (TREM2) have been reported3–6 and 16 pathogenic mutations in these genes have been detected in Italian FTD patients and explain part of the disease heritability (Table S1, Fig. 1). Here, PRNP is linked to frontotemporal dementia.