A polycystin-centric view of cyst formation and disease: The polycystins revisited

Albert C M Ong, Peter C Harris

Research output: Contribution to journalArticle

91 Scopus citations

Abstract

It is 20 years since the identification of PKD1, the major gene mutated in autosomal dominant polycystic kidney disease (ADPKD), followed closely by the cloning of PKD2. These major breakthroughs have led in turn to a period of intense investigation into the function of the two proteins encoded, polycystin-1 and polycystin-2, and how defects in either protein lead to cyst formation and nonrenal phenotypes. In this review, we summarize the major findings in this area and present a current model of how the polycystin proteins function in health and disease.

Original languageEnglish (US)
Pages (from-to)699-710
Number of pages12
JournalKidney International
Volume88
Issue number4
DOIs
StatePublished - Oct 3 2015

Keywords

  • ADPKD
  • PKD1
  • PKD2
  • polycystin-1
  • polycystin-2
  • TRPP2

ASJC Scopus subject areas

  • Nephrology

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