Polycystins and primary cilia: primers for cell cycle progression.
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.
Zhou
Jing
J
Renal Division, Department of Medicine, Brigham and Women's Hospital and Harvard Medical School, Boston, Massachusetts 02115, USA. zhou@rics.bwh.harvard.edu
D000818:Animals; D020013:Calcium Signaling / Q000502:physiology; D002453:Cell Cycle / Q000502:physiology*; D002923:Cilia / Q000502:physiology*; D019204:GTP-Binding Proteins / Q000502:physiology; D006801:Humans; D040542:Mechanotransduction, Cellular / Q000502:physiology; D007690:Polycystic Kidney Diseases / Q000503:physiopathology; D050396:TRPP Cation Channels / Q000502:physiology*
D050396:TRPP Cation Channels; D019204:GTP-Binding Proteins