NDT Advance Access originally published online on September 26, 2008
Nephrology Dialysis Transplantation 2008 23(12):3773-3775; doi:10.1093/ndt/gfn533
| ||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||
© The Author [2008]. Published by Oxford University Press on behalf of ERA-EDTA. All rights reserved. For Permissions, please e-mail: journals.permissions@oxfordjournals.org
Does TNF-
enhance cystogenesis in ADPKD?*
Division of Nephrology, Cliniques Universitaires Saint-Luc and Université catholique de Louvain Medical School, B-1200 Brussels, Belgium
Correspondence and offprint requests to: Prof. Y. Pirson, Service de Néphrologie, Cliniques Universitaires Saint-Luc, Av. Hippocrate, 10, B-1200 BRUXELLES. Tel: +32-2-7641857; Fax: +32-2-7642836; E-mail: yves.pirson@uclouvain.be
Keywords: autosomal dominant polycystic kidney disease; autosomal recessive polycystic kidney disease; ethanercept; FIP-2; TNF-
| The first 150 words of the full text of this article appear below. |
| Unravelling nongenetic factors contributing to cystogenesis in ADPKD |
|---|
Autosomal dominant polycystic kidney disease (ADPKD) is caused by mutation in either PKD1 (85%) or PKD2 (15%) genes. The resulting disease phenotypes are very similar, except that renal disease is typically less severe in PKD2 families [1].
The protein products of PDK1 and PKD2, polycystin-1 (PC-1) and polycystin-2 (PC-2), are membrane proteins that probably form a functional complex [1]. PC-1 is regarded as a receptor for an unidentified ligand while PC-2 has significant homology to the transient receptor potential (TRP) family of store-operated calcium channels and is likely to function similarly as a non-selective calcium channel [1–3]. Like many other proteins implicated in renal cystic diseases, PC-1 and PC-2 are located in the primary cilium, a single hair-like organelle projecting from the surface of most mammalian cells. In tubular epithelial cells, the cilium projects into the lumen and is thought to act as
Exploring TNF- -mediated pathway as an enhancer of ADPKD
|
|---|
Revealing a pathway connecting TNF- signalling, polycystins and cystogenesis
|
|---|
Weighing potential clinical implications of this work
Take-home message