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A mechanistic approach to inherited polycystic kidney disease

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Abstract

There are approximately six and a half million people, of the estimated world population of six billion, with inherited polycystic kidney disease. Polycystic kidney diseases have a broad spectrum of associated findings that distinguish and define them as specific disease states. The dysregulation of renal tubular epithelial cell biology, including cell polarity, cell signaling, proliferation and apoptosis, basement membrane and matrix abnormalities, and fluid transport, has been postulated to contribute to cystogenesis. Evidence is currently accumulating that supports an association of the primary cilium and basal body, as well as the focal adhesion assembly, with polycystic kidney diseases. Renal cystogenesis may be the result of a disruption of a critical feedback loop that regulates tissue morphology based on the epithelial cell environment.

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Acknowledgements

The bibliography is restrained by space limitations, and the authors apologize to the scientists whose work has not been cited. The authors would like to thank Drs. Vincent Torres, Clark D. West, and Lisa Guay-Woodford for their careful review of the manuscript and constructive comments. This work was supported by the following grants to J.J.B. from NIH (DK61458 and CA103486), LAM Foundation, and TS Alliance.

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  1. Division of Nephrology and Hypertension, Cincinnati Children’s Hospital Medical Center, Cincinnati, OH 45229-3039, USA

    John J. Bissler & Bradley P. Dixon

  2. Children’s Hospital Research Foundation, ML 7022, 3333 Burnet Avenue, Cincinnati, OH 45229-3039, USA

    John J. Bissler

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  1. John J. Bissler
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  2. Bradley P. Dixon
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Correspondence to John J. Bissler.

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Bissler, J.J., Dixon, B.P. A mechanistic approach to inherited polycystic kidney disease. Pediatr Nephrol 20, 558–566 (2005). https://doi.org/10.1007/s00467-004-1665-z

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  • DOI: https://doi.org/10.1007/s00467-004-1665-z

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