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

C. Boutin, P. Labedan, J. Dimidschstein, F. Richard, H. Cremer, P. Andre, Y. Yang, M. Montcouquiol, A. M. Goffinet, F. Tissir
Proceedings of the National Academy of Sciences. 2014-07-14; 111(30): E3129-E3138
DOI: 10.1073/pnas.1404988111

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1. 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.

Boutin C(1), Labedan P(2), Dimidschstein J(3), Richard F(4), Cremer H(4), André
P(5), Yang Y(5), Montcouquiol M(6), Goffinet AM(1), Tissir F(7).

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; .

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.

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


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