It remains to be seen whether an understanding of the opposing roles of ENPP1 in the suppression of ectopic mineralization and maintenance of bone mass will unlock the treatment of CKD-MBD patients, but efforts to this end are currently being explored, including a natural history study in calciphylaxis patients demonstrating that their plasma PPi levels are comparable to levels observed in GACI patients and correlate with mortality. Here, ENPP1 is linked to chronic kidney disease.