Renal handling of phosphate in vivo and in vitro by the X-linked hypophosphatemic male mouse: evidence for a defect in the brush border membrane.
Renal handling of phosphate in vivo and in vitro by the X-linked hypophosphatemic male mouse: evidence for a defect in the brush border membrane.
复制标题
X连锁低磷血症雄性小鼠体内和体外肾脏对磷酸盐的处理：刷状缘膜缺陷的证据。
作者：
H. Tenenhouse;C. Scriver;R. Mcinnes;F. Glorieux
X连锁低磷血症雄性小鼠体内和体外肾脏对磷酸盐的处理：刷状缘膜缺陷的证据。我们在低磷血症的雄性突变小鼠（Hyp/Y）中检查了肾磷酸盐的体外转运和体内重吸收，该小鼠携带一种被认为与导致人类X连锁低磷血症的基因同源的基因。正常磷酸盐血症的雄性同窝小鼠(+/Y)用作对照。 Hyp/Y 小鼠中磷酸盐的排泄分数增加（0.351 ± 0.073 [平均值 ± SD, N=7] 对比 +/Y 小鼠中的 0.210 ± 0.0885 [N = 15] [P 32 P-磷酸盐，在培养基中为 0.1 和 1.0 mM，通过皮质或髓质切片以及细胞内无机和有机池的标记 切片观察到一级动力学，并且在两种小鼠品系中没有显着差异。饮食中钙的调节影响了这两种菌株的观察结果，但没有揭示它们之间的差异。从小鼠肾皮质匀浆制备的纯化刷状缘膜囊泡通过钠依赖性、砷酸盐抑制的转运系统积累磷酸盐。磷酸盐的总吸收（钠依赖性加上 +/Y囊泡的钠独立性为每60秒488±23皮摩尔/毫克蛋白质。 （平均值±标准差）和 Hyp/Y 囊泡每 60 秒 302 ± 10 pmoles/mg 蛋白质（P 体内和体外数据表明，X 连锁表型中磷酸盐的净跨上皮转运受损是净通量内在缺陷的结果 刷状缘膜上的磷酸盐。
Renal handling of phosphate in vivo and in vitro by the X-linked hypophosphatemic male mouse: Evidence for a defect in the brush border membrane. We have examined renal phosphate transport in vitro and reabsorption in vivo in hypophosphatemic, male mutant mice (Hyp/Y) carrying a gene believed to be homologous with that responsible for X-linked hypophosphatemia in man. Nor-mophosphatemic male littermates (+/Y) were used as controls. Fractional excretion of phosphate in Hyp/Y mice is increased (0.351 ± 0.073 [mean ± SD, N=7] vs. 0.210 ± 0.0885 [N = 15] in +/Y mice [P 32 P-phosphate, at 0.1 and 1.0 mM in the medium, by slices of cortex or medulla, and labeling of intracellular inorganic and organic pools of slices observed first-order kinetics and was not significantly different in the two strains of mice. Modulation of calcium in the diet influenced these observations in both strains but did not disclose differences between them. Purified brush border membrane vesicles prepared from mouse kidney cortex homoge-nates accumulated phosphate by a sodium-dependent, arsenate-inhibited transport system. Total uptake of phosphate (sodium-dependent plus sodium-independent) by +/Y vesicles was 488 ± 23 pmoles/mg of protein per 60 sec. (mean ± SD) and 302 ± 10 pmoles/mg of protein per 60 sec by Hyp/Y vesicles (P in vivo and in vitro data indicate that impaired net transepithelial transport of phosphate in the X-linked phenotype is the result of an intrinsic defect in net flux of phosphate at the brush border membrane.