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Permanent URL to this publication: http://dx.doi.org/10.5167/uzh-52806

Terryn, S; Ho, A; Beauwens, R; Devuyst, O (2011). Fluid transport and cystogenesis in autosomal dominant polycystic kidney disease. Biochimica et Biophysica Acta, 1812(10):1314-1321.

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Abstract

Autosomal dominant polycystic kidney disease (ADPKD) is the most frequent inherited nephropathy. The development and enlargement of cysts in ADPKD requires tubular cell proliferation, abnormalities in the extracellular matrix and transepithelial fluid secretion. Multiple studies have suggested that fluid secretion across ADPKD cyst-lining cells is driven by the transepithelial secretion of chloride, mediated by the apical CFTR channel and specific basolateral transporters. The whole secretory process is stimulated by increased levels of cAMP in the cells, probably reflecting modifications in the intracellular calcium homeostasis and abnormal stimulation of the vasopressin V2 receptor. This review will focus on the pathophysiology of fluid secretion in ADPKD cysts, starting with classic, morphological and physiological studies that were followed by investigations of the molecular mechanisms involved and therapeutic trials targeting these pathways in cellular and animal models and ADPKD patients. This article is part of a Special Issue entitled: Polycystic Kidney Disease.

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19 citations in Web of Science®
21 citations in Scopus®
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Additional indexing

Item Type:Journal Article, not refereed, further contribution
Communities & Collections:04 Faculty of Medicine > Institute of Physiology
07 Faculty of Science > Institute of Physiology
DDC:570 Life sciences; biology
Language:English
Date:2011
Deposited On:11 Jan 2012 11:29
Last Modified:27 Nov 2013 18:53
Publisher:Elsevier
ISSN:0006-3002
Publisher DOI:10.1016/j.bbadis.2011.01.011
PubMed ID:21255645

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