Abstract
For quite some time the field of polycystic kidney disease has led a life at the fringe of kidney research, but with the cloning of the PKD1 and many other genes this situation has dramatically changed. Polycystic kidney disease often is a syndromic disease affecting a variety of organs in addition to the kidney. Most of the proteins involved in polycystic kidney disease have been localized to the primary cilium, an extension at the apical membrane of renal tubular epithelial cells, which may serve chemo- and mechanosensory functions. It is speculated that primary cilia and their associated proteins play a role in determining the proper tubular geometry.
Keywords: metanephric kidney, mutations, polycystin, glycoproteins, endoplasmic reticulum, nephrocystins, nphp gene, bardet-biedl proteins, cilia, chemosensors
Current Molecular Medicine
Title: New Developments in the Field of Cystic Kidney Diseases
Volume: 5 Issue: 5
Author(s): Ralph Witzgall
Affiliation:
Keywords: metanephric kidney, mutations, polycystin, glycoproteins, endoplasmic reticulum, nephrocystins, nphp gene, bardet-biedl proteins, cilia, chemosensors
Abstract: For quite some time the field of polycystic kidney disease has led a life at the fringe of kidney research, but with the cloning of the PKD1 and many other genes this situation has dramatically changed. Polycystic kidney disease often is a syndromic disease affecting a variety of organs in addition to the kidney. Most of the proteins involved in polycystic kidney disease have been localized to the primary cilium, an extension at the apical membrane of renal tubular epithelial cells, which may serve chemo- and mechanosensory functions. It is speculated that primary cilia and their associated proteins play a role in determining the proper tubular geometry.
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Cite this article as:
Witzgall Ralph, New Developments in the Field of Cystic Kidney Diseases, Current Molecular Medicine 2005; 5 (5) . https://dx.doi.org/10.2174/1566524054553496
DOI https://dx.doi.org/10.2174/1566524054553496 |
Print ISSN 1566-5240 |
Publisher Name Bentham Science Publisher |
Online ISSN 1875-5666 |
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