Skip Navigation

This Article
Right arrow Full Text Freely available
Right arrow FREE Full Text (PDF) Freely available
Right arrow Alert me when this article is cited
Right arrow Alert me if a correction is posted
Services
Right arrow Email this article to a friend
Right arrow Similar articles in this journal
Right arrow Similar articles in ISI Web of Science
Right arrow Similar articles in PubMed
Right arrow Alert me to new issues of the journal
Right arrow Add to My Personal Archive
Right arrow Download to citation manager
Right arrow Search for citing articles in:
ISI Web of Science (7)
Right arrowRequest Permissions
Right arrow Disclaimer
Google Scholar
Right arrow Articles by Joly, D.
Right arrow Articles by Knebelmann, B.
Right arrow Search for Related Content
PubMed
Right arrow PubMed Citation
Right arrow Articles by Joly, D.
Right arrow Articles by Knebelmann, B.
Social Bookmarking
Add to CiteULike   Add to Connotea   Add to Del.icio.us  
What's this?

Nephrol Dial Transplant (2003) 18: 1689-1692
© 2003 European Renal Association-European Dialysis and Transplant Association

Editorial Comment

Ciliary function of polycystins: a new model for cystogenesis

Dominique Joly, Aurélie Hummel, Antonella Ruello and Bertrand Knebelmann

INSERM U 507, Service de Néphrologie, Hôpital Necker-Enfants Malades et Université Paris V, Paris, France

Correspondence and offprint requests to: Dominique Joly, INSERM U 507, Service de Néphrologie, Hôpital Necker-Enfants Malades et Université Paris V, 161 Rue de Sevres, Paris, France. Email: joly@necker.fr

Keywords: autosomal dominant polycystic kidney disease; primary cilia; polycystins cation channels

The first 150 words of the full text of this article appear below.

Introduction

Autosomal dominant polycystic kidney disease results from loss-of-function mutations in either polycystin-1 (Pc-1) or polycystin-2 (Pc-2). These transmembrane proteins directly interact through cytosolic domains of their C-termini. Pc-1 has been implicated in cell–extracellular matrix (ECM) interactions at focal adhesion contacts, but also in cell–cell interactions at tight junctions, adherens junctions and desmosomes [1]. Pc-2, located to the plasma membrane and/or to the endoplasmic reticulum [2,3], was shown to increase the intracytosolic calcium from both ER stocks and extracellular milieu [4]. The variability of polycystin localization within the cell may be explained by differences in antibody characteristics, cell type, degree of confluence, developmental stage, but also raises the hypothesis of multiple biological functions [1]. The large extracellular domain of Pc-1 is thought to transduce signals from the environment into the cells, through Pc-1-dependent signalling pathways and through activation of PC-2 calcium . . . [Full Text of this Article]

Renal primary cilia

Polycystins are located in renal primary cilia

Ciliary function of polycystins
Cilia and cystogenesis


Add to CiteULike CiteULike   Add to Connotea Connotea   Add to Del.icio.us Del.icio.us    What's this?


This article has been cited by other articles:


Home page
 Nephrol Dial TransplantHome page
E. Fischer, L. Gresh, A. Reimann, and M. Pontoglio
Cystic kidney diseases: learning from animal models
Nephrol. Dial. Transplant., November 1, 2004; 19(11): 2700 - 2702.
[Full Text] [PDF]