Abnormal regulation of renal 25-hydroxyvitamin D-1 alpha-hydroxylase activity in the X-linked hypophosphatemic mouse.
Abnormal regulation of renal 25-hydroxyvitamin D-1 alpha-hydroxylase activity in the X-linked hypophosphatemic mouse.
复制标题
X 连锁低磷血症小鼠肾脏 25-羟基维生素 D-1 α-羟化酶活性的异常调节。
DOI：
10.1172/jci110783
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发表时间：
1983
期刊：
影响因子：
--
通讯作者：
Drezner,MK
中科院分区：
文献类型：
--
作者：
Lobaugh,B;Drezner,MK
在X连锁低磷酸盐血症佝偻病（XLH）和X连锁低磷酸盐血症小鼠（Hyp-mice）（人类疾病的小鼠同源物）患者中怀疑维生素D代谢异常。我们比较了25（OH）D-1 α-羟化酶活性在Hyp-mouse肾脏，在正常和磷酸盐耗尽的小鼠肾脏。断奶的正常小鼠和Hyp-mice小鼠喂食0.6%的磷饲料;磷酸盐耗尽的小鼠喂食0.02%的磷饲料。在8-10周龄时，Hyp（3.35 +/- 0.12 mg/dl）和磷酸盐耗竭小鼠（3.83 +/- 0.56）的血清磷值无显著差异。尽管磷酸盐消耗的幅度相似，但是25（OH）D-1 α-羟化酶活性的最大水平是不同的：与正常小鼠相比，磷酸盐耗尽的小鼠肾脏活性显著增加（17.04 +/-0.104 vs. 4. 96 +/-0.23 fmol 1，25（OH）2D 3产生/mg肾脏/min），而Hyp-mouse肾脏具有四倍的较小增量（8.18 +/-0.62）。这些数据表明，磷酸盐消耗是（C57 BL 6 J）小鼠中25（OH）D-1 α-羟化酶活性的有效刺激物。此外，结果表明，25（OH）D-1 α-羟化酶活性的异常调节在Hyp-mouse中是明显的。
Abnormal vitamin D metabolism has been suspected in patients with X-linked hypophosphatemic rickets (XLH) and X-linked hypophosphatemic mice (Hyp-mice), the murine homologue of the human disease. We compared 25(OH)D-1 alpha-hydroxylase activity in the Hyp-mouse kidney to that in normal and phosphate-depleted mouse kidney. Weanling normal and Hyp-mice were fed a 0.6% phosphorus diet; phosphate-depleted mice received a 0.02% phosphorus diet. At 8-10 wk of age the serum phosphorus values in Hyp (3.35 +/- 0.12 mg/dl) and phosphate-depleted mice (3.83 +/- 0.56) were not significantly different. Despite the similar magnitude of phosphate depletion, however, the maximum levels of 25(OH)D-1 alpha-hydroxylase activity were disparate: phosphate-depleted mouse kidney had profoundly increased activity compared to normal (17.04 +/- .104 vs. 4.96 +/- 0.23 fmol 1,25(OH)2D3 produced/mg kidney per min) while Hyp-mouse kidney had a fourfold lesser increment (8.18 +/- 0.62). These data indicate that phosphate depletion is a potent stimulus of 25(OH)D-1 alpha-hydroxylase activity in the (C57BL6J) mouse. Moreover, the results show that abnormal regulation of 25(OH)D-1 alpha-hydroxylase activity is manifest in the Hyp-mouse.