TY - JOUR
T1 - A dual role for planar cell polarity genes in ciliated cells
AU - Boutin, Camille
AU - Labedan, Paul
AU - Dimidschstein, Jordane
AU - Richard, Fabrice
AU - Cremer, Harold
AU - André, Philipp
AU - Yang, Yingzi
AU - Montcouquiol, Mireille
AU - Goffinet, Andre M.
AU - Tissir, Fadel
PY - 2014
Y1 - 2014
N2 - 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.
AB - 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.
UR - http://www.scopus.com/inward/record.url?scp=84904994416&partnerID=8YFLogxK
U2 - 10.1073/pnas.1404988111
DO - 10.1073/pnas.1404988111
M3 - Article
C2 - 25024228
AN - SCOPUS:84904994416
SN - 0027-8424
VL - 111
SP - E3129-E3138
JO - Proceedings of the National Academy of Sciences of the United States of America
JF - Proceedings of the National Academy of Sciences of the United States of America
IS - 30
ER -