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Proc Natl Acad Sci U S A. 2014 Jul 29;111(30):E3129-38. doi: 10.1073/pnas.1404988111. Epub 2014 Jul 14.

A dual role for planar cell polarity genes in ciliated cells.

Author information

1
Institute of Neuroscience, Developmental Neurobiology, Université catholique de Louvain, B1200 Brussels, Belgium;
2
PLab, B1200 Brussels, Belgium;
3
Institut de Recherches en Biologie Humaine et Moléculaire, Université Libre de Bruxelles, 1070 Brussels, Belgium;
4
Aix-Marseille Université, Centre National de la Recherche Scientifique, Institut de Biologie du Développement de Marseille Luminy, Unité Mixte de Recherche 7288, 13288 Marseille, France;
5
Developmental Genetics Section, Genetic Disease Research Branch, National Human Genome Research Institute, Bethesda, MD 20892; and.
6
Planar Polarity and Plasticity Group, Institut National de la Santé et de la Recherche Médicale U862, Neurocenter Magendie, 33077 Bordeaux, France.
7
Institute of Neuroscience, Developmental Neurobiology, Université catholique de Louvain, B1200 Brussels, Belgium; fadel.tissir@uclouvain.be.

Abstract

In the nervous system, cilia dysfunction perturbs the circulation of the cerebrospinal fluid, thus affecting neurogenesis and brain homeostasis. A role for planar cell polarity (PCP) signaling in the orientation of cilia (rotational polarity) and ciliogenesis is established. However, whether and how PCP regulates cilia positioning in the apical domain (translational polarity) in radial progenitors and ependymal cells remain unclear. By analysis of a large panel of mutant mice, we show that two PCP signals are operating in ciliated cells. The first signal, controlled by cadherin, EGF-like, laminin G-like, seven-pass, G-type receptor (Celsr) 2, Celsr3, Frizzled3 (Fzd3) and Van Gogh like2 (Vangl2) organizes multicilia in individual cells (single-cell polarity), whereas the second signal, governed by Celsr1, Fzd3, and Vangl2, coordinates polarity between cells in both radial progenitors and ependymal cells (tissue polarity). Loss of either of these signals is associated with specific defects in the cytoskeleton. Our data reveal unreported functions of PCP and provide an integrated view of planar polarization of the brain ciliated cells.

PMID:
25024228
PMCID:
PMC4121795
DOI:
10.1073/pnas.1404988111
[Indexed for MEDLINE]
Free PMC Article

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