کد مقاله کد نشریه سال انتشار مقاله انگلیسی نسخه تمام متن
1905170 1534691 2011 5 صفحه PDF دانلود رایگان
عنوان انگلیسی مقاله ISI
Polycystins, focal adhesions and extracellular matrix interactions
موضوعات مرتبط
علوم زیستی و بیوفناوری بیوشیمی، ژنتیک و زیست شناسی مولکولی سالمندی
پیش نمایش صفحه اول مقاله
Polycystins, focal adhesions and extracellular matrix interactions
چکیده انگلیسی

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.

Research highlights
► Changes in extracellular matrix composition and cell–matrix interactions have long been associated with ADPKD but remain understudied and poorly understood.
► Further examination of cell–matrix interaction will contribute to our understanding of cystogenesis as well as help clarify the basis of extrarenal manifestations of ADPKD that include abdominal hernia, intracranial aneurysm, gastrointestinal cysts, and cardiac valvular defects, conditions that are commonly associated with altered extracellular matrix integrity.
► In addition to their proposed role in primary cilia, polycystins might play a direct role in extracellular matrix sensing or metabolism by acting as mechanosensors in focal adhesion complexes.

ناشر
Database: Elsevier - ScienceDirect (ساینس دایرکت)
Journal: Biochimica et Biophysica Acta (BBA) - Molecular Basis of Disease - Volume 1812, Issue 10, October 2011, Pages 1322–1326
نویسندگان
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