News from the cyst: insights into polycystic kidney disease.

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.

journal_name

J Nephrol

journal_title

Journal of nephrology

authors

Wagner CA

subject

Has Abstract

pub_date

2008-01-01 00:00:00

pages

14-6

issue

1

eissn

1121-8428

issn

1724-6059

journal_volume

21

pub_type

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