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Biochim Biophys Acta. 2011 Oct;1812(10):1322-6. doi: 10.1016/j.bbadis.2011.03.003. Epub 2011 Mar 9.

Polycystins, focal adhesions and extracellular matrix interactions.

Author information

  • 1Massachusetts General Hospital, MA, USA. idrummon@receptor.mgh.harvard.edu

Abstract

Polycystic kidney disease is the most common heritable disease in humans. In addition to epithelial cysts in the kidney, liver and pancreas, patients with autosomal dominant polycystic kidney disease (ADPKD) also suffer from abdominal hernia, intracranial aneurysm, gastrointestinal cysts, and cardiac valvular defects, conditions often associated with altered extracellular matrix production or integrity. Despite more than a decade of work on the principal ADPKD genes, PKD1 and PKD2, questions remain about the basis of cystic disease and the role of extracellular matrix in ADPKD pathology. This review explores the links between polycystins, focal adhesions, and extracellular matrix gene expression. These relationships suggest roles for polycystins in cell-matrix mechanosensory signaling that control matrix production and morphogenesis. This article is part of a Special Issue entitled: Polycystic Kidney Disease.

Copyright © 2011 Elsevier B.V. All rights reserved.

PMID:
21396443
[PubMed - indexed for MEDLINE]
PMCID:
PMC3132319
Free PMC Article
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