ArticleJournal of dental research2018
Defective Mineralization in X-Linked Hypophosphatemia Dental Pulp Cell Cultures.
Article in Journal of dental research, 2018. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 22 papers.
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Who cites it
22 citing papers in PubMed, 33 citations in OpenAlex.
- Knockdown of MEPE Promotes Cranial Defect Repair and Activates the cAMP/PKA Signaling Pathway.Biochemical genetics · 2026Article
- Article
- Genetic reduction or deletion of osteopontin in osteomalacicJBMR plus · 2025Article
- A novel phex gene variant causes non-syndromic tooth agenesis.BMC oral health · 2025Article
- Advancing patient evidence in XLH (APEX): rationale and design of a real-world XLH global data unification program.Frontiers in endocrinology · 2025Article
- Inherited phosphate and pyrophosphate disorders: New insights and novel therapies changing the oral health landscape.Journal of the American Dental Association (1939) · 2024Review
- Characteristics of oral health of patients with X-linked hypophosphatemia: case reports and literature review.BDJ open · 2024Review
- X-Linked Hypophosphatemia: Does Targeted Therapy Modify Dental Impairment?Journal of clinical medicine · 2023Review
- Dental manifestations and treatment of hypophosphatemic rickets: A case report and review of literature.BDJ open · 2023Article
- Dental health of patients with X-linked hypophosphatemia: A controlled study.Frontiers in oral health · 2023Article
- Dental health of pediatric patients with X-linked hypophosphatemia (XLH) after three years of burosumab therapy.Frontiers in endocrinology · 2022Article
- Vitamin D and Phosphate Interactions in Health and Disease.Advances in experimental medicine and biology · 2022Article
- Burden of disease and clinical targets in adult patients with X-linked hypophosphatemia. A comprehensive review.Osteoporosis international : a journal established as result of cooperation between the European Foundation for Osteoporosis and the National Osteoporosis Foundation of the USA · 2021Review
- Characterization of Oral Health Status in Chilean Patients with X-Linked Hypophosphatemia.Calcified tissue international · 2021Observational
- Mineralized tissues in hypophosphatemic rickets.Pediatric nephrology (Berlin, Germany) · 2020Review
- Article
- Multidisciplinary patient care in X-linked hypophosphatemic rickets: one challenge, many perspectives.Wiener medizinische Wochenschrift (1946) · 2020Review
- Development of Enthesopathies and Joint Structural Damage in a Murine Model of X-Linked Hypophosphatemia.Frontiers in cell and developmental biology · 2020Article
- Impact of Early Conventional Treatment on Adult Bone and Joints in a Murine Model of X-Linked Hypophosphatemia.Frontiers in cell and developmental biology · 2020Article
- Priming Dental Pulp Stem Cells from Human Exfoliated Deciduous Teeth with Fibroblast Growth Factor-2 Enhances Mineralization Within Tissue-Engineered Constructs Implanted in Craniofacial Bone Defects.Stem cells translational medicine · 2019Article
Corrections and comments
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Authors and funding
11 authors at 6 institutions in 3 countries.
Funding
No grant is acknowledged in the PubMed record.
Abstract
X-linked hypophosphatemia (XLH) is a skeletal disease caused by inactivating mutations in the PHEX gene. Mutated or absent PHEX protein/enzyme leads to a decreased serum phosphate level, which cause mineralization defects in the skeleton and teeth (osteomalacia/odontomalacia). It is not yet altogether clear whether these manifestations are caused solely by insufficient circulating phosphate availability for mineralization or also by a direct, local intrinsic effect caused by impaired PHEX activity. Here, we evaluated the local role of PHEX in a 3-dimensional model of extracellular matrix (ECM) mineralization. Dense collagen hydrogels were seeded either with human dental pulp cells from patients with characterized PHEX mutations or with sex- and age-matched healthy controls and cultured up to 24 d using osteogenic medium with standard phosphate concentration. Calcium quantification, micro-computed tomography, and histology with von Kossa staining for mineral showed significantly lower mineralization in XLH cell-seeded scaffolds, using nonparametric statistical tests. While apatitic mineralization was observed along collagen fibrils by electron microscopy in both groups, Raman microspectrometry indicated that XLH cells harboring the PHEX mutation produced less mineralized scaffolds having impaired mineral quality with less carbonate substitution and lower crystallinity. In the XLH cultures, immunoblotting revealed more abundant osteopontin (OPN), dentin matrix protein 1 (DMP1), and matrix extracellular phosphoglycoprotein (MEPE) than controls, as well as the presence of fragments of these proteins not found in controls, suggesting a role for PHEX in SIBLING protein degradation. Immunohistochemistry revealed altered OPN and DMP1 associated with an increased alkaline phosphatase staining in the XLH cultures. These results are consistent with impaired PHEX activity having local ECM effects in XLH. Future treatments for XLH should target both systemic and local manifestations.
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