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News from the cyst: insights into polycystic kidney disease


Wagner, C A (2008). News from the cyst: insights into polycystic kidney disease. Journal of Nephrology, 21(1):14-16.

Abstract

Autosomal recessive and autosomal dominant polycystic kidney disease (ADPKD and ARPKD) are 2 major variants of inherited diseases leading to the cystic degeneration of the kidney and other organs. Primary cilia have moved into the focus of research as it is becoming increasingly clear that dysfunction of this special cell organelle represents the main underlying mechanism of disease. At the same time, we also learn that the primary cilium plays an important role in the regulation of cell proliferation and transport processes along the nephron in the healthy kidney. Three recent publications implicate 2 novel players in ciliogenesis and cyst formation: the von Hippel Lindau tumor suppressor gene (pVHL) and collectrin.

Abstract

Autosomal recessive and autosomal dominant polycystic kidney disease (ADPKD and ARPKD) are 2 major variants of inherited diseases leading to the cystic degeneration of the kidney and other organs. Primary cilia have moved into the focus of research as it is becoming increasingly clear that dysfunction of this special cell organelle represents the main underlying mechanism of disease. At the same time, we also learn that the primary cilium plays an important role in the regulation of cell proliferation and transport processes along the nephron in the healthy kidney. Three recent publications implicate 2 novel players in ciliogenesis and cyst formation: the von Hippel Lindau tumor suppressor gene (pVHL) and collectrin.

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4 citations in Web of Science®
3 citations in Scopus®
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Additional indexing

Item Type:Journal Article, refereed, further contribution
Communities & Collections:04 Faculty of Medicine > Center for Integrative Human Physiology
04 Faculty of Medicine > Institute of Physiology
07 Faculty of Science > Institute of Physiology
Dewey Decimal Classification:570 Life sciences; biology
610 Medicine & health
Language:English
Date:February 2008
Deposited On:09 Dec 2008 19:12
Last Modified:21 Nov 2017 13:36
Publisher:Wichtig Editore
ISSN:1121-8428
PubMed ID:18264930

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