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NDT Advance Access originally published online on November 29, 2007
Nephrology Dialysis Transplantation 2008 23(4):1157-1165; doi:10.1093/ndt/gfm763
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© The Author [2007]. Published by Oxford University Press on behalf of ERA-EDTA. All rights reserved. For Permissions, please e-mail: journals.permissions@oxfordjournals.org


Overexpression of PKD2 in the mouse is associated with renal tubulopathy

Stéphane Burtey1,*, Marta Riera1,*,**, Emilie Ribe1, Petra Pennekamp2, Edith Passage1, Roselyne Rance1, Bernd Dworniczak2 and Michel Fontés1

1 INSERM UMR 491, Medical Genetics and Development, IPHM, Faculté de Médecine de la Timone. 27 Bd. J. Moulin. 13385 Marseille cedex 5. France 2 Institute for Human Genetics, University Hospital Muenster, Vesaliusweg 12-14, 48149 Muenster, Germany

Michel Fontès, Génétique Médicale et Développement, INSERM UMR 491 Faculté de Médecine de la Timone, 27 Bd. J. Moulin 13385 Marseille cedex 5, France. Tel: +33-(0)4-91-25-71-59; E-mail: michel.fontes{at}medecine.univ-mrs.fr



  Abstract

Polycystin-2 (PC-2), a cation channel of the Trp family, is involved in autosomal dominant polycystic kidney disease (ADPKD) type 2 (ADPKD2). This protein has recently been localized to the primary cilium where its channel function seems to be involved in a mechanosensory phenomenon. However, its biological function is not totally understood, especially in tubule formation. In the present paper, we describe a mouse model for human PC-2 overexpression, obtained by inserting a human bacterial artificial chromosome (BAC) containing the PKD2 gene. Three lines were generated, expressing different levels of PKD2. One line, PKD2-Y, has been explored in more detail and we will present physiological and molecular exploration of these transgenic animals. Our data demonstrate that transgenic animals older than 12 months present tubulopathy with proteinuria and failure to concentrate urine. Moreover, the kidney cortex has been found disorganized. Finally, we observe that extracellular matrix protein expression is downregulated in these animals. In conclusion, overexpression of human PKD2 leads to anomalies in tubular function, probably due to abnormalities in tubule morphogenesis.

Keywords: abnormal extracellular matrix; fibronectin; PKD2; tubular dysfunction

* The first two authors have contributed equally to the work.

** Present address: Laboratory de fisiopatologia renal. Instituto de resercha val hebron. 08035 Barcelona.

Received for publication: 7. 5.07
Accepted in revised form: 28. 9.07


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