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This Article Full Text Full Text (PDF) All Versions of this Article: 578/3/641    most recent jphysiol.2006.119024v1 Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Download to citation manager Citing Articles Citing Articles via HighWire Citing Articles via Google Scholar Google Scholar Articles by Kiselyov, K. Articles by Muallem, S. Search for Related Content PubMed PubMed Citation Articles by Kiselyov, K. Articles by Muallem, S. Related Collections Review articles

¹Department of Biological Sciences, University of Pittsburgh, Pittsburgh, PA 15260, USA ²Department of Physiology, UT South-Western Medical Center at Dallas, Dallas, TX 75390, USA

Many human diseases are caused by mutations in ion channels. Dissecting the pathogenesis of these ‘channelopathies’ has yielded important insights into the regulation of vital biological processes by ions and has become a productive tool of modern ion channel biology. One of the best examples of a synergism between the clinical and basic science aspects of a modern biological topic is cystic fibrosis. Not only did the identification of the ion channel mutated in cystic fibrosis pinpoint the root cause of this disease, but it also has significantly advanced our understanding of basic biological processes as diverse as protein folding and epithelial fluid and electrolyte secretion. The list of confirmed ‘channelopathies’ is growing and several members of the TRP family of ion channels have been implicated in human diseases such as mucolipidosis type IV (MLIV), autosomal dominant polycystic kidney disease (ADPKD), familial focal segmental glomerulosclerosis (FSG), hypomagnesemia with secondary hypocalcaemia (HSH), and several forms of cancer. Analysing pathogenesis of the diseases linked to TRP dysregulation provides an exciting means of identifying novel functions of TRP channels.

(Received 14 August 2006; accepted after revision 23 November 2006; first published online 30 November 2006)
Corresponding author K. Kiselyov: Department of Biological Sciences, University of Pittsburgh, 4249 Fifth Avenue, Pittsburgh, PA 15260, USA. Email: kiselyov{at}pitt.edu

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