Defective planar cell polarity in polycystic kidney disease.
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.
Fischer
Evelyne
E
Gene Expression and Disease Unit, Centre National de la Recherche Scientifique (CNRS) FRE 2850, Pasteur Institute, 25 rue du Docteur Roux, 75724 Paris Cedex 15, France.
Legue
Emilie
E
Doyen
Antonia
A
Nato
Faridabano
F
Nicolas
Jean-François
JF
Torres
Vicente
V
Yaniv
Moshe
M
Pontoglio
Marco
M
D000818:Animals; D016764:Cell Polarity; D004195:Disease Models, Animal; D051539:Hepatocyte Nuclear Factor 1-beta / Q000235:genetics; D007684:Kidney Tubules / Q000187:drug effects / Q000473:pathology*; D051379:Mice; D008817:Mice, Mutant Strains; D008938:Mitosis; D009088:Mucoproteins / Q000378:metabolism; D007690:Polycystic Kidney Diseases / Q000235:genetics / Q000473:pathology*; D051381:Rats; D017207:Rats, Sprague-Dawley; D008941:Spindle Apparatus / Q000502:physiology; D013629:Tamoxifen / Q000494:pharmacology; D058949:Uromodulin
D009088:Mucoproteins; C548613:Umod protein, mouse; D058949:Uromodulin; D013629:Tamoxifen; D051539:Hepatocyte Nuclear Factor 1-beta