Valenti Giovanna, Tamma Grazia
Department of Biosciences, Biotechnologies, and Biopharmaceutics, University of Bari, Bari, Italy.
Department of Biosciences, Biotechnologies, and Biopharmaceutics, University of Bari, Bari, Italy.
Handb Clin Neurol. 2021;181:249-259. doi: 10.1016/B978-0-12-820683-6.00018-X.
Vasopressin is the key hormone involved in water conservation and regulation of water balance, essential for life. In the renal collecting duct, vasopressin binds to the V2 receptor, increasing water permeability through activation of aquaporin-2 redistribution to the luminal membrane. This mechanism promotes rapid water reabsorption, important for immediate survival; however, only recently it has become clear that long-term adverse effects are associated with alterations of the vasopressin-aquaporin-2 pathway, leading to several syndromes associated with water balance disorders. The kidney resistance to the vasopressin action may cause severe dehydration for patients and, conversely, nonosmotic release of vasopressin is associated with water retention and increasing the circulatory blood volume. This chapter discusses the relevance of the altered vasopressin-aquaporin-2 pathway in some diseases associated with water balance disorders, including congenital nephrogenic diabetes insipidus, syndrome of inappropriate secretion of antidiuretic hormone, nephrogenic syndrome of inappropriate antidiuresis, and autosomal dominant polycystic kidney disease. The emerging picture suggests that targeting the vasopressin-AQP2 axis can provide therapeutic benefits in those patients.
血管加压素是参与水潴留和水平衡调节的关键激素，对生命至关重要。在肾集合管中，血管加压素与V2受体结合，通过激活水通道蛋白-2向管腔膜的重新分布来增加水通透性。这种机制促进快速的水重吸收，对即刻生存很重要；然而，直到最近才清楚，血管加压素-水通道蛋白-2途径的改变会产生长期不良影响，导致几种与水平衡紊乱相关的综合征。肾脏对血管加压素作用的抵抗可能会导致患者严重脱水，相反，血管加压素的非渗透性释放与水潴留和循环血容量增加有关。本章讨论了血管加压素-水通道蛋白-2途径改变在一些与水平衡紊乱相关疾病中的相关性，包括先天性肾性尿崩症、抗利尿激素分泌不当综合征、肾性抗利尿不当综合征和常染色体显性多囊肾病。新出现的情况表明，针对血管加压素-AQP2轴可为这些患者带来治疗益处。
