Inactivating mutations in the genes encoding enzymes involved in PPi homeostasis result in rare hereditary calcification disorders which include pseudoxanthoma elasticum (PXE, OMIM 264800), generalized arterial calcification of infancy (GACI, OMIM 208000), arterial calcification due to CD73 deficiency (ACDC, OMIM 211800), and Hutchinson–Gilford Progeria Syndrome (HGPS, OMIM 176670). The gene discussed is NT5E; the disease is Pseudoxanthoma elasticum.