作者
Shiguang Liu, Jianping Zhou, Wen Tang, Xi Jiang, David W Rowe, L Darryl Quarles
发表日期
2006/7
期刊
American Journal of Physiology-Endocrinology and Metabolism
卷号
291
期号
1
页码范围
E38-E49
出版商
American Physiological Society
简介
Inactivating mutations of the PHEX (phosphate-regulating gene with homologies to endopeptidases on the X chromosome) endopeptidase, the disease-causing gene in X-linked hypophosphatemia (XLH), results in increased circulating levels of fibroblastic growth factor-23 (FGF23), a bone-derived phosphaturic factor. To determine the causal role of FGF23 in XLH, we generated a combined Fgf23-deficient enhanced green fluorescent protein (eGFP) reporter and Phex-deficient Hyp mouse model (Fgf23+/−/Hyp). eGFP expression was expressed in osteocytes embedded in bone that exhibited marked upregulation of eGFP in response to Phex deficiency and in CD31-positive cells in bone marrow venules that expressed low eGFP levels independently of Phex. In bone marrow stromal cells (BMSCs) derived from Fgf23−/−/Hyp mice, eGFP expression was also selectively increased in osteocyte-like cells within …
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S Liu, J Zhou, W Tang, X Jiang, DW Rowe, LD Quarles - American Journal of Physiology-Endocrinology and …, 2006