Article
Osteopontin and the dento-osseous pathobiology of X-linked hypophosphatemia.
Bone - 1 Feb 2017
Boukpessi Tchilalo, Hoac Betty, Coyac Benjamin R, Leger Thibaut, Garcia Camille, Wicart Philippe, Whyte Michael P, Glorieux Francis H, Linglart Agnès, Chaussain Catherine, McKee Marc D
Abstract excerpt
Seven young patients with X-linked hypophosphatemia (XLH, having inactivating PHEX mutations) were discovered to accumulate osteopontin (OPN) at the sites of defective bone mineralization near osteocytes - the so-called hallmark periosteocytic (lacunar) "halos" of XLH. OPN was also localized in the pericanalicular matrix extending beyond the osteocyte lacunae, as well as in the hypomineralized matrix of tooth...
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