Defective planar cell polarity in polycystic kidney disease

Nat Genet. 2006 Jan;38(1):21-3. doi: 10.1038/ng1701. Epub 2005 Dec 11.

Abstract

Morphogenesis involves coordinated proliferation, differentiation and spatial distribution of cells. We show that lengthening of renal tubules is associated with mitotic orientation of cells along the tubule axis, demonstrating intrinsic planar cell polarization, and we demonstrate that mitotic orientations are significantly distorted in rodent polycystic kidney models. These results suggest that oriented cell division dictates the maintenance of constant tubule diameter during tubular lengthening and that defects in this process trigger renal tubular enlargement and cyst formation.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Animals
  • Cell Polarity*
  • Disease Models, Animal
  • Hepatocyte Nuclear Factor 1-beta / genetics
  • Kidney Tubules / drug effects
  • Kidney Tubules / pathology*
  • Mice
  • Mice, Mutant Strains
  • Mitosis
  • Mucoproteins / metabolism
  • Polycystic Kidney Diseases / genetics
  • Polycystic Kidney Diseases / pathology*
  • Rats
  • Rats, Sprague-Dawley
  • Spindle Apparatus / physiology
  • Tamoxifen / pharmacology
  • Uromodulin

Substances

  • Mucoproteins
  • Umod protein, mouse
  • Uromodulin
  • Tamoxifen
  • Hepatocyte Nuclear Factor 1-beta