Proof-of-principle for enhanced dentoalveolar mineralization using exogenous tissue-nonspecific alkaline phosphatase in the <i>Hyp</i> mouse model of X-linked hypophosphatemia.

ABSTRACT: X-linked hypophosphatemia (XLH) is caused by mutations in the PHEX gene, which leads to increased levels of fibroblast growth factor 23 and hypophosphatemia, contributing to rickets, osteomalacia, and dentoalveolar defects, including severe dentin hypomineralization, thin cementum, and alveolar bone osteomalacia. Current XLH treatment options appear to have limited efficacy on dentoalveolar tissues, suggesting underlying disease mechanisms that remain unchecked. Increased production of inorganic pyrophosphate (PP_i) and osteopontin (OPN), both mineralization inhibitors, has been posited to contribute to mineralization defects in XLH. The enzyme, tissue-nonspecific alkaline phosphatase (TNAP) reduces PP_i levels via hydrolysis and inactivates OPN by dephosphorylat

SUBMITTER: Phanrungsuwan A 

PROVIDER: S-EPMC12599311 | biostudies-literature | 2025 Dec

REPOSITORIES: biostudies-literature