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Am J Physiol Renal Physiol (August 22, 2007). doi:10.1152/ajprenal.00275.2007
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Submitted on June 14, 2007
Accepted on August 17, 2007

Polycystic kidney disease and renal injury repair: common pathways, fluid flow and the function of polycystin-1

Thomas Weimbs1*

1 Department of Molecular, Cellular, and Developmental Biology, University of California Santa Barbara, Santa Barbara, California, United States

* To whom correspondence should be addressed. E-mail: weimbs{at}lifesci.ucsb.edu.

The root cause for most cases of autosomal-dominant polycystic kidney disease (ADPKD) are mutations in the polycystin-1 (PC1) gene. While PC1 has been implicated in a perplexing variety of protein interactions and signaling pathways, it is unknown what its normal function is and why its disruption leads to proliferation of renal epithelial cells. Recent results suggest that PC1 is involved in mechanotransduction by primary cilia measuring the degree of luminal fluid flow. PC1 has also recently been shown to regulate the mTOR and STAT6 pathways. These two pathways are normally dormant in the healthy kidney but are activated in response to injury and appear to drive a proliferative repair response. This review is developing the idea that a critical function of polycystin-1 and primary cilia in the adult kidney may be to sense renal injury by detecting changes in luminal fluid flow and to trigger proliferation. Constitutive activation of these pathways in ADPKD would lead to the futile attempt to repair a non-existing injury, resulting in cyst growth. The existence of many known cellular and molecular similarities between renal repair and ADPKD supports this model.




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