While the function of proteins encoded by SLC20A2, XPR1, PDGFRB, PDGFB, and JAM2 have previously been described, and have offered some insights into their roles in PFBC progression [8–10], the function and activity of the myogenesis-regulating glycosidase (MYORG) encoded by MYORG remain unknown. This evidence concerns the gene MYORG and bilateral striopallidodentate calcinosis.