Journal Article

The ciliary flow sensor and polycystic kidney disease

Fruzsina Kotsis, Christopher Boehlke and E. Wolfgang Kuehn

in Nephrology Dialysis Transplantation

Published on behalf of European Renal Association - European Dialysis and Transplant Assoc

Volume 28, issue 3, pages 518-526
Published in print March 2013 | ISSN: 0931-0509
Published online January 2013 | e-ISSN: 1460-2385 | DOI: http://dx.doi.org/10.1093/ndt/gfs524

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Since the discovery that proteins mutated in different forms of polycystic kidney disease (PKD) are tightly associated with primary cilia, strong efforts have been made to define the role of this organelle in the pathogenesis of cyst formation. Cilia are filiform microtubular structures, anchored in the basal body and extending from the apical membrane into the tubular lumen. Early work established that cilia act as flow sensors, eliciting calcium transients in response to bending, which involve the two proteins mutated in autosomal dominant PKD (ADPKD), polycystin-1 and -2. Loss of cilia alone is insufficient to cause cyst formation. Nevertheless, a large body of evidence links flow sensing by cilia to aspects relevant for cyst formation such as cell polarity, Stat6- and mammalian target of rapamycin signalling. This review summarizes the current literature on cilia and flow sensing with respect to PKD and discusses how these findings intercalate with different aspects of cyst formation.

Keywords: cilia; ADPKD; mechanosensation; polarity; mTOR; calcium

Journal Article.  5484 words.  Illustrated.

Subjects: Nephrology

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