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Clinical and Experimental Nephrology
Published in: Clinical and Experimental Nephrology 3/2018

Open Access 01-06-2018 | Invited review article

Activation of AQP2 water channels without vasopressin: therapeutic strategies for congenital nephrogenic diabetes insipidus

Authors: Fumiaki Ando, Shinichi Uchida

Published in: Clinical and Experimental Nephrology | Issue 3/2018

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Abstract

Congenital nephrogenic diabetes insipidus (NDI) is characterized by defective urine concentrating ability. Symptomatic polyuria is present from birth, even with normal release of the antidiuretic hormone vasopressin by the pituitary. Over the last two decades, the aquaporin-2 (AQP2) gene has been cloned and the molecular mechanisms of urine concentration have been gradually elucidated. Vasopressin binds to the vasopressin type II receptor (V2R) in the renal collecting ducts and then activates AQP2 phosphorylation and trafficking to increase water reabsorption from urine. Most cases of congenital NDI are caused by loss-of-function mutations to V2R, resulting in unresponsiveness to vasopressin. In this article, we provide an overview of novel therapeutic molecules of congenital NDI that can activate AQP2 by bypassing defective V2R signaling with a particular focus on the activators of the calcium and cAMP signaling pathways.
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Metadata
Title
Activation of AQP2 water channels without vasopressin: therapeutic strategies for congenital nephrogenic diabetes insipidus
Authors
Fumiaki Ando
Shinichi Uchida
Publication date
01-06-2018
Publisher
Springer Singapore
Published in
Clinical and Experimental Nephrology / Issue 3/2018
Print ISSN: 1342-1751
Electronic ISSN: 1437-7799
DOI
https://doi.org/10.1007/s10157-018-1544-8

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