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NDT Advance Access originally published online on October 30, 2007
Nephrology Dialysis Transplantation 2007 22(12):3367-3370; doi:10.1093/ndt/gfm426
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© The Author [2007]. Published by Oxford University Press on behalf of ERA-EDTA. All rights reserved. For Permissions, please email: journals.permissions@oxfordjournals.org


Molecular pathogenesis of ADPKD and development of targeted therapeutic options

Oxana Ibraghimov-Beskrovnaya

Cell Biology, Genzyme Corporation, 5 Mountain Road, Framingham, MA, USA

Correspondence and offprint requests to: Oxana Ibraghimov-Beskrovnaya, Genzyme Corporation, 5 Mountain Road, Framingham, MA 01701-9322, USA. Email: oxana.beskrovnaya@genzyme.com

Keywords: apoptosis; cAMP; cell cycle; cilium; polycystic kidney disease

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



   Introduction
 
Autosomal dominant polycystic kidney disease (ADPKD) is a common genetic disease characterized by formation and progressive enlargement of cysts in kidneys, liver and other organs, leading to end stage renal disease by the fifth decade [1]. Mutations in the PKD1 gene encoding polycystin-1 are responsible for ~85% of ADPKD cases, while mutations in the PKD2 gene cause ~15% of ADPKD cases with a less severe phenotype. Autosomal recessive polycystic kidney disease (ARPKD) affects newborns and results from mutations in the PKHD1 gene encoding fibrocystin [2]. Regardless of the genetic defect underlying PKD, cystic epithelia seem to display common abnormalities. Cellular mechanisms of cystogenesis have been studied for decades and suggest that cystic epithelia are characterized by a secretory phenotype with increased proliferation, apoptosis, loss of cellular differentiation and polarity. Considerable progress towards understanding the molecular pathogenesis of cyst formation has been made since the cloning of . . . [Full Text of this Article]



   Increased proliferation and apoptosis in PKD
 


   cAMP activated pathways
 


   mTOR activation
 


   Cilia and cell cycle disruption
 


   Conclusions
 

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