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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.

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Figure 1: Programmed pattern of cell division in elongating renal tubules.
Figure 2: Mitotic orientation in tubular cells.


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We are grateful to B. Mateescu for the H3pS10 antibody and to A. Blanchard for the aqp2 antibody. We thank E. Perret, P. Roux and M. Delarue for assistance and advice, and F. Terzi and J. Weitzman for helpful discussions and for critical reading of the manuscript. E.F. is a fellow of the Association pour l'Information et la Recherche sur les maladies Rénales Génétiques (AIRG). This work was supported by the PKD Foundation, Genzyme Corporation and La Ligue contre le Cancer, as well as by grants (to E.L. and J.F.N.) from Cells into Organs (EU Framework 6 project LSHM-CT2003-504468), EurostemCell (EU Framework 6 project LHSB-CT2003-503005) and the Association Française contre les Myopathies (AFM).

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Supplementary information

Supplementary Fig. 1

Tubular cell proliferation in newborn mice. (PDF 708 kb)

Supplementary Fig. 2

Tubular luminal diameter in precystic pck rats. (PDF 66 kb)

Supplementary Fig. 3

Onset of mitotic distortion precedes tubular dilation in mutant mice. (PDF 147 kb)

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Fischer, E., Legue, E., Doyen, A. et al. Defective planar cell polarity in polycystic kidney disease. Nat Genet 38, 21–23 (2006).

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