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首页> 外文期刊>American Journal of Physiology >Salt and acid-base metabolism in claudin-16 knockdown mice: impact for the pathophysiology of FHHNC patients
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Salt and acid-base metabolism in claudin-16 knockdown mice: impact for the pathophysiology of FHHNC patients

机译：claudin-16基因敲除小鼠的盐和酸碱代谢：对FHHNC患者病理生理的影响

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SAalt and acid-base metabolism in claudin-16 knockdown mice: impact for the pathophysiology of FHHNC patients. Am J Physiol Renal Physiol 295: F1641-F1647, 2008. First published September 10, 2008; doi:10.1152/ajprenal.90388.2008.-Claudin-16 is defective in familial hypomagnesemia with hypercalciuria and nephrocalcinosis (FHHNC). Claudin-16 knockdown (CLDN16 KD) mice show reduced cation selectivity in the thick ascending limb. The defect leads to a collapse of the lumen-positive diffusion voltage, which drives Ca2+ and Mg2+ absorption. Because of the reduced tight junction permeability ratio for Na+ over Cl~, We^proposed a backleak of NaCl into the lumen. Systemic analysis had revealed lower blood pressure and a moderately increased plasma aldosterone concentration. In this study, we measured the amiloride-sensitive equivalent short-circuit current in isolated, perfused collecting ducts and found it increased by fivefold in CLDN16 KD mice compared with wild-type (WT) mice. Amiloride treatment unmasked renal Na+ loss in the thick ascending limb of the nephron. Under amiloride treatment, CLDN16 KD mice developed hyponatremia and the renal fractional excretion of Na+ was twofold higher in CLDN16 KD compared with WT mice. The loss of claudin-16 also resulted in increased urinary flow, reduced HCOaT excretion, and lower urine pH. We conclude that perturbation in salt and acid-base metabolism in CLDN16 KD mice has its origin in the defective cation permselectivity of the thick ascending limb of the nephron. This study has contributed to the still incomplete understanding of the symptoms of FHHNC patients
机译：claudin-16基因敲除小鼠的SAALT和酸碱代谢：对FHHNC患者病理生理的影响。 Am J Physiol Renal Physiol 295：F1641-F1647，2008年。2008年9月10日首次发布。 doi：10.1152 / ajprenal.90388.2008.-Claudin-16在家族性低镁血症中伴有高钙尿症和肾钙化病（FHHNC）缺陷。克劳丁16基因敲低（CLDN16 KD）小鼠显示出浓厚的上升肢体中阳离子选择性降低。缺陷导致管腔正扩散电压崩溃，从而驱动Ca2 +和Mg2 +吸收。由于Na +相对于Cl-的紧密连接渗透率降低，我们提出了NaCl向管腔的回漏。全身分析显示血压降低，血浆醛固酮浓度适度增加。在这项研究中，我们测量了离体，灌注收集管中阿米洛利敏感的等效短路电流，发现与野生型（WT）小鼠相比，CLDN16 KD小鼠的阿米洛利等效短路电流增加了五倍。阿米洛利治疗掩盖了肾的厚上升肢中肾脏Na +的流失。在阿米洛利治疗下，CLDN16 KD小鼠发生低钠血症，与野生型小鼠相比，CLDN16 KD的肾脏Na +排泄分数高出两倍。 claudin-16的丢失还导致尿流量增加，HCOaT排泄减少和尿液pH值降低。我们得出的结论是，CLDN16 KD小鼠的盐和酸碱代谢紊乱的根源在于肾的厚上升肢的阳离子渗透选择性不良。这项研究有助于对FHHNC患者的症状仍不完全了解

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