Journal
NATURE REVIEWS NEPHROLOGY
Volume 6, Issue 9, Pages 530-538Publisher
NATURE PUBLISHING GROUP
DOI: 10.1038/nrneph.2010.97
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Funding
- ANR 2005 cardiovasculaire-obesite-diabete
- ANR 2008 du gene a la physiopathologie
- Association for information and research on genetic kidney disease France
- Fondation del Duca
- Fondation de la recherche medicale
- Fondation de France
- Fondation de recherche sur l'hypertension arterielle
- Federation pour la recherche sur le cerveau
- Societe Generale AM
- Universite de Nice-Sophia Antipolis
- CNRS
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Autosomal dominant polycystic kidney disease is a common disorder, affecting approximately one in 1,000 individuals. This disease is characterized by the presence of renal and extrarenal cysts, as well as by cardiovascular abnormalities, including hypertension and intracranial aneurysms. Mutations in the PKD1 gene account for 85% of cases, whereas mutations in PKD2 account for the remaining 15% of cases. Findings from the past 10 years indicate that polycystins, the products of the PKD genes, have a key role in renal and vascular mechanosensory transduction. In the primary cilium of renal, nodal, and endothelial cells, polycystins are proposed to act as flow sensors. In addition, the ratio of polycystin-1 to polycystin-2 regulates pressure sensing in arterial myocytes. In this Review, we summarize the data indicating that polycystins are key molecules in mechanotransduction. Moreover, we discuss the role of nucleotide release and autocrine and/or paracrine purinergic signaling in both fluid flow and pressure responses. Finally, we discuss the possible role of altered mechanosensory transduction in the etiology of polycystic kidney disease.
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