Sitara Despina
Department of Developmental Biology, Harvard School of Dental Medicine, Boston, MA 02115, USA.
Ann N Y Acad Sci. 2007 Nov;1116:485-93. doi: 10.1196/annals.1402.021. Epub 2007 Jul 23.
Phosphate homeostasis is mostly regulated through humoral factors exerting direct or indirect effects on transporter proteins located in the intestine and kidney. Fibroblast growth factor 23 (FGF-23) is a major phosphate-regulating molecule, which can affect both renal and intestinal phosphate uptake to influence overall mineral ion homeostasis. We have found that Fgf-23 gene knockout mice (Fgf-23(-/-)) develop hyperphosphatemia that consequently leads to abnormal bone mineralization, and severe soft tissue calcifications. On the contrary, FGF-23 transgenic mice develop hypophosphatemia and produce rickets-like features in the mutant bone. Further studies using our Fgf-23(-/-) mice have identified an inverse correlation between Fgf-23, and vitamin D or NaPi2a; genomic elimination of either vitamin D or NaPi2a activities from Fgf-23(-/-) mice could reverse severe hyperphosphatemia to hypophosphatemia, and consequently could alter skeletal mineralization, suggesting that regulation of phosphate homeostasis in Fgf-23(-/-) mice is vitamin D- and NaPi2a-mediated process.
磷稳态主要通过体液因子对位于肠道和肾脏的转运蛋白产生直接或间接影响来调节。成纤维细胞生长因子23(FGF - 23)是一种主要的磷调节分子,它可以影响肾脏和肠道对磷的摄取,从而影响整体矿物质离子稳态。我们发现,Fgf - 23基因敲除小鼠(Fgf - 23(-/-))会出现高磷血症,进而导致骨矿化异常和严重的软组织钙化。相反,FGF - 23转基因小鼠会出现低磷血症,并在突变骨骼中出现类似佝偻病的特征。使用我们的Fgf - 23(-/-)小鼠进行的进一步研究发现,Fgf - 23与维生素D或NaPi2a之间呈负相关;从Fgf - 23(-/-)小鼠中基因消除维生素D或NaPi2a的活性可以将严重的高磷血症逆转至低磷血症,从而改变骨骼矿化,这表明Fgf - 23(-/-)小鼠中磷稳态的调节是由维生素D和NaPi2a介导的过程。