Article
Hexa-D-arginine treatment increases 7B2•PC2 activity in hyp-mouse osteoblasts and rescues the HYP phenotype.
Journal of bone and mineral research : the official journal of the American Society for Bone and Mineral Research - 1 Jan 2013
Yuan Baozhi, Feng Jian Q, Bowman Stephen, Liu Ying, Blank Robert D, Lindberg Iris, Drezner Marc K
Abstract excerpt
Inactivating mutations of the "phosphate regulating gene with homologies to endopeptidases on the X chromosome" (PHEX/Phex) underlie disease in patients with X-linked hypophosphatemia (XLH) and the hyp-mouse, a murine homologue of the human disorder. Although increased serum fibroblast growth factor 23 (FGF-23) underlies the HYP phenotype, the mechanism(s) by which PHEX mutations inhibit FGF-23 degradation and/or...
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