Polycystins and primary cilia: primers for cell cycle progression

Annu Rev Physiol. 2009;71:83-113. doi: 10.1146/annurev.physiol.70.113006.100621.

Abstract

Polycystins are a family of eight-transmembrane proteins united by sequence homology. The name stems from the identification of mutations in genes encoding polycystin-1 and -2 in polycystic kidney diseases. This review discusses recent topics in polycystin research, with a focus on the role of polycystin-1 and polycystin-2 in primary cilia and the cell cycle. Polycystins appear to play key roles during development, but a major question is their function in mature organs. Their roles in primary cilia, shear stress sensation, alteration of intracellular calcium, and planar cell polarity are examined. The third-hit hypothesis of polycystic kidney disease is discussed.

Publication types

  • Research Support, N.I.H., Extramural
  • Review

MeSH terms

  • Animals
  • Calcium Signaling / physiology
  • Cell Cycle / physiology*
  • Cilia / physiology*
  • GTP-Binding Proteins / physiology
  • Humans
  • Mechanotransduction, Cellular / physiology
  • Polycystic Kidney Diseases / physiopathology
  • TRPP Cation Channels / physiology*

Substances

  • TRPP Cation Channels
  • GTP-Binding Proteins