title: A dual role for planar cell polarity genes in ciliated cells.
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.
authors.affiliation: Institute of Neuroscience, Developmental Neurobiology, Université catholique de Louvain, B1200 Brussels, Belgium;
authors.forename: Camille
authors.initials: C
authors.lastname: Boutin
authors.affiliation: PLab, B1200 Brussels, Belgium;
authors.forename: Paul
authors.initials: P
authors.lastname: Labedan
authors.affiliation: Institut de Recherches en Biologie Humaine et Moléculaire, Université Libre de Bruxelles, 1070 Brussels, Belgium;
authors.forename: Jordane
authors.initials: J
authors.lastname: Dimidschstein
authors.affiliation: 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;
authors.forename: Fabrice
authors.initials: F
authors.lastname: Richard
authors.affiliation: 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;
authors.forename: Harold
authors.initials: H
authors.lastname: Cremer
authors.affiliation: Developmental Genetics Section, Genetic Disease Research Branch, National Human Genome Research Institute, Bethesda, MD 20892; and.
authors.forename: Philipp
authors.initials: P
authors.lastname: André
authors.affiliation: Developmental Genetics Section, Genetic Disease Research Branch, National Human Genome Research Institute, Bethesda, MD 20892; and.
authors.forename: Yingzi
authors.initials: Y
authors.lastname: Yang
authors.affiliation: Planar Polarity and Plasticity Group, Institut National de la Santé et de la Recherche Médicale U862, Neurocenter Magendie, 33077 Bordeaux, France.
authors.forename: Mireille
authors.initials: M
authors.lastname: Montcouquiol
authors.affiliation: Institute of Neuroscience, Developmental Neurobiology, Université catholique de Louvain, B1200 Brussels, Belgium;
authors.forename: Andre M
authors.initials: AM
authors.lastname: Goffinet
authors.affiliation: Institute of Neuroscience, Developmental Neurobiology, Université catholique de Louvain, B1200 Brussels, Belgium; fadel.tissir@uclouvain.be.
authors.forename: Fadel
authors.initials: F
authors.lastname: Tissir
mesh_terms: D000818:Animals; D016764:Cell Polarity / Q000502:physiology*; D002923:Cilia / Q000235:genetics / Q000378:metabolism; D003599:Cytoskeleton / Q000235:genetics / Q000378:metabolism*; D004805:Ependyma / Q000166:cytology / Q000378:metabolism*; D051379:Mice; D018345:Mice, Knockout; D009419:Nerve Tissue Proteins / Q000235:genetics / Q000378:metabolism*; D055495:Neurogenesis / Q000502:physiology*; D015398:Signal Transduction / Q000502:physiology*
chemical_list: D009419:Nerve Tissue Proteins