Osteopontin and the dento-osseous pathobiology of X-linked hypophosphatemia

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• 作者：Tchilalo Boukpessi^a ; ^b ; ^c ; ^{Tchilalo.boukpessi@parisdescartes.fr} ; Betty Hoac^a ; Benjamin R. Coyac^a ; ^b ; Thibaut Leger^d ; Camille Garcia^d ; Philippe Wicart^e ; Michael P. Whyte^f ; Francis H. Glorieux^g ; Agn&egrave ; s Linglart^h ; Catherine Chaussain^b ; ^c ; ¹ ; Marc D. McKee^a ; ⁱ ; ¹ ; ^{marc.mckee@mcgill.ca}
• 关键词：PHEX ; Mineralization ; Rickets ; Bone ; Dentin ; Osteocytic halos ; Osteomalacia
• 刊名：Bone
• 出版年：2017
• 出版时间：February 2017
• 年：2017
• 卷：95
• 期：Complete
• 页码：151-161
• 全文大小：1508 K
• 卷排序：95

文摘

Osteomalacia and odontomalacia in XLH have increased osteopontin in the matrix. Osteopontin in XLH bone and dentin matrix co-localizes with hypomineralization sites. Periosteocytic lesions (hypomineralized halos) of XLH show osteopontin accumulation. Osteopontin co-localizes with a hypomineralized canalicular network. Mineralization-inhibiting matrix osteopontin may contribute to the XLH phenotype.