Genetic reduction or deletion of osteopontin in osteomalacic <i>Hyp</i> mice fails to ameliorate dentoalveolar defects.

ABSTRACT: X-linked hypophosphatemia (XLH) is caused by inactivating mutations in PHEX, leading to disturbed mineral metabolism, rickets, and osteomalacia. Dentoalveolar defects affecting dentin, cementum, and alveolar bone, contribute to dental abscesses and periodontal breakdown. Limited efficacy of current therapies for ameliorating XLH-associated dentoalveolar effects suggests mechanisms of disease that remain unchecked. Increased production and abnormal distribution of osteopontin (OPN) is thought to contribute to XLH-associated mineralization defects. Osteopontin is a secreted phosphoprotein and mineralization inhibitor, and a substrate of PHEX. To investigate the potential role of OPN in the pathology of XLH-associated dentoalveolar defects, a genetic approach was used to ablate or dimi

SUBMITTER: Phanrungsuwan A 

PROVIDER: S-EPMC12642872 | biostudies-literature | 2025 Dec

REPOSITORIES: biostudies-literature