Dishevelled stabilization by the ciliopathy protein rpgrip1l is essential for planar cell polarity

Alexia Mahuzier, Helori-Mael Gaudé, Valentina Grampa, Isabelle Anselme, Flora Silbermann, Margot Leroux-Berger, Delphine Delacour, Jerome Ezan, Mireille Montcouquiol, Sophie Saunier, Sylvie Schneider-Maunoury, Christine Vesque
J Cell Biol. 2012-08-27; 198(5): 927-940
DOI: 10.1083/jcb.201111009

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1. J Cell Biol. 2012 Sep 3;198(5):927-40. doi: 10.1083/jcb.201111009. Epub 2012 Aug
27.

Dishevelled stabilization by the ciliopathy protein Rpgrip1l is essential for
planar cell polarity.

Mahuzier A(1), Gaudé HM, Grampa V, Anselme I, Silbermann F, Leroux-Berger M,
Delacour D, Ezan J, Montcouquiol M, Saunier S, Schneider-Maunoury S, Vesque C.

Author information:
(1)Centre National de la Recherche Scientifique UMR 7622, Institut National de la
Santé et de la Recherche Médicale U969, 75005 Paris, France.

Cilia are at the core of planar polarity cellular events in many systems.
However, the molecular mechanisms by which they influence the polarization
process are unclear. Here, we identify the function of the ciliopathy protein
Rpgrip1l in planar polarity. In the mouse cochlea and in the zebrafish floor
plate, Rpgrip1l was required for positioning the basal body along the planar
polarity axis. Rpgrip1l was also essential for stabilizing dishevelled at the
cilium base in the zebrafish floor plate and in mammalian renal cells. In rescue
experiments, we showed that in the zebrafish floor plate the function of Rpgrip1l
in planar polarity was mediated by dishevelled stabilization. In cultured cells,
Rpgrip1l participated in a complex with inversin and nephrocystin-4, two
ciliopathy proteins known to target dishevelled to the proteasome, and, in this
complex, Rpgrip1l prevented dishevelled degradation. We thus uncover a ciliopathy
protein complex that finely tunes dishevelled levels, thereby modulating planar
cell polarity processes.

DOI: 10.1083/jcb.201111009
PMCID: PMC3432770
PMID: 22927466 [Indexed for MEDLINE]


Auteurs Bordeaux Neurocampus