X连锁低磷血症小鼠中肾25-羟维生素D-1α-羟化酶活性的异常调节
Abnormal regulation of renal 25-hydroxyvitamin D-1 alpha-hydroxylase activity in the X-linked hypophosphatemic mouse.
作者信息
Lobaugh B, Drezner M K
出版信息
J Clin Invest. 1983 Feb;71(2):400-3. doi: 10.1172/jci110783.
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.
X连锁低磷性佝偻病（XLH）患者及X连锁低磷小鼠（Hyp小鼠，人类该疾病的小鼠同源模型）被怀疑存在维生素D代谢异常。我们比较了Hyp小鼠肾脏中25(OH)D - 1α - 羟化酶活性与正常及低磷小鼠肾脏中的该酶活性。将断乳的正常小鼠和Hyp小鼠喂食含0.6%磷的饮食；低磷小鼠则喂食含0.02%磷的饮食。在8 - 10周龄时，Hyp小鼠（3.35±0.12mg/dl）和低磷小鼠（3.83±0.56）的血清磷值无显著差异。然而，尽管磷缺乏程度相似，但25(OH)D - 1α - 羟化酶活性的最高水平却不同：与正常小鼠相比，低磷小鼠肾脏的活性显著增加（17.04±0.104对4.96±0.23 fmol 1,25(OH)2D3产生量/mg肾脏每分钟），而Hyp小鼠肾脏的增加幅度则小四倍（8.18±0.62）。这些数据表明，磷缺乏是（C57BL6J）小鼠中25(OH)D - 1α - 羟化酶活性的有力刺激因素。此外，结果显示Hyp小鼠中存在25(OH)D - 1α - 羟化酶活性的异常调节。
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