Title :

Impaired mineral quality in dentin in X-linked hypophosphatemia

Author(s) :

Coyac, Benjamin [Auteur]
Falgayrac, Guillaume [Auteur]
Université de Lille
Marrow Adiposity & Bone Lab - Adiposité Médullaire et Os - ULR 4490 [MABLab]
Penel, Guillaume [Auteur]
Marrow Adiposity & Bone Lab - Adiposité Médullaire et Os - ULR 4490 [MABLab]
Schmitt, Alain [Auteur]
Institut Cochin [IC UM3 (UMR 8104 / U1016)]
Schinke, Thorsten [Auteur]
Linglart, Agnès [Auteur]
Signalisation Hormonale, Physiopathologie Endocrinienne et Métabolique
Hôpital Bicêtre [AP-HP, Le Kremlin-Bicêtre]
Mckee, Marc [Auteur]
McGill University = Université McGill [Montréal, Canada]
Chaussain, Catherine [Auteur]
Université Paris Descartes - Faculté de Chirurgie Dentaire [UPD5 Odontologie]
Bardet, Claire [Auteur]
Université Paris Descartes - Faculté de Chirurgie Dentaire [UPD5 Odontologie]

Journal title :

Connective Tissue Research

Pages :

91-96

Publisher :

Taylor & Francis

Publication date :

2018-05-10

ISSN :

0300-8207

HAL domain(s) :

Sciences du Vivant [q-bio]

English abstract : [en]

X-linked hypophosphatemia (XLH) is a skeletal disorder arising from mutations in the PHEX gene, transmitted in most cases as an X-linked dominant trait. PHEX deficiency leads to renal phosphate wasting and hypophosphatemia, ...
Show more >X-linked hypophosphatemia (XLH) is a skeletal disorder arising from mutations in the PHEX gene, transmitted in most cases as an X-linked dominant trait. PHEX deficiency leads to renal phosphate wasting and hypophosphatemia, as well as impaired mineralization of bone and dentin, resulting in severe skeletal and dental complications. Dentin mineralization defects appear as characteristic, large interglobular spaces resulting from the lack of fusion of calculospherites in the circumpulpal region during the mineralization process. Here, we examined changes in the composition and structure of dentin using Raman spectroscopy on XLH human teeth, and using transmission electron microscopy on the dentin of Hyp mice (the murine model of XLH). The dentin of patients with XLH showed changes in the quality of the apatitic mineral, with greater carbonate substitution and lower crystallinity compared to the dentin of age-matched control teeth. In addition, ultrastructural analysis by transmission electron microscopy revealed a major disorganization of the peri- and intertubular structure of the dentin, with odontoblast processes residing within an unmineralized matrix sheath in the Hyp mouse. Taken together, these results indicate that like for bone and tooth cementum, there are impaired mineral quality and matrix changes in XLH dentin reflecting high sensitivity to systemic serum phosphate levels and possibly other local changes in the dentin matrix.Show less >

Language :

Anglais

Popular science :

Non

Collections :

• Marrow Adiposity & Bone Lab (MABLab) - ULR 4490

Source :

Harvested from HAL