Article
Impaired mineral quality in dentin in X-linked hypophosphatemia.
Connective tissue research - 1 Dec 2018
Coyac Benjamin R, Falgayrac Guillaume, Penel Guillaume, Schmitt Alain, Schinke Thorsten, Linglart Agnès, McKee Marc D, Chaussain Catherine, Bardet Claire
Abstract excerpt
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...
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