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Cystic kidney diseases and planar cell polarity signaling

Authors

  • RL Bacallao,

    1. Division of Nephrology, Indiana University School of Medicine, Indianapolis, IN, USA
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  • H McNeill

    Corresponding author
    1. Samuel Lunenfeld Research Institute, Mt. Sinai Hospital, Toronto, Canada
    2. Department of Molecular Genetics, University of Toronto, Toronto, Ontario, Canada
      Helen McNeill,
      Room 884, SLRI, Mt. Sinai Hospital,
      600 University Avenue,
      Toronto, Ontario, Canada,
      M5G 1X5.
      Tel.: 416-586-8267;
      fax: 416-586-8588;
      e-mail: mcneill@lunenfeld.ca
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Helen McNeill,
Room 884, SLRI, Mt. Sinai Hospital,
600 University Avenue,
Toronto, Ontario, Canada,
M5G 1X5.
Tel.: 416-586-8267;
fax: 416-586-8588;
e-mail: mcneill@lunenfeld.ca

Abstract

Renal cystic diseases are a major clinical concern as they are the most common genetic cause of end-stage renal disease. While many of the genes causing cystic disease have been identified in recent years, knowing the molecular nature of the mutations has not clarified the mechanisms underlying cyst formation. Recent research in model organisms has suggested that cyst formation may be because of defective planar cell polarity (PCP) and/or ciliary defects. In this review, we first outline the clinical features of renal cystic diseases and then discuss current research linking our understanding of cystic kidney disease to PCP and cilia.

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