Celsr1 coordinates the planar polarity of vestibular hair cells during inner ear development.

TitleCelsr1 coordinates the planar polarity of vestibular hair cells during inner ear development.
Publication TypeJournal Article
Year of Publication2017
AuthorsDuncan, JS, Stoller, ML, Francl, AF, Tissir, F, Devenport, D, Deans, MR
JournalDev Biol
Date Published2017 03 15
KeywordsAnimals, Behavior, Animal, Cell Polarity, Ear, Inner, Epithelium, Gene Deletion, Hair Cells, Vestibular, Mice, Knockout, Organ of Corti, Phenotype, Receptors, G-Protein-Coupled, Signal Transduction, Stereocilia

<p>Vestibular hair cells of the inner ear are specialized receptors that detect mechanical stimuli from gravity and motion via the deflection of a polarized bundle of stereocilia located on their apical cell surfaces. The orientation of stereociliary bundles is coordinated between neighboring cells by core PCP proteins including the large adhesive G-protein coupled receptor Celsr1. We show that mice lacking Celsr1 have vestibular behavioral phenotypes including circling. In addition, we show that Celsr1 is asymmetrically distributed at cell boundaries between hair cells and neighboring supporting cells in the developing vestibular and auditory sensory epithelia. In the absence of Celsr1 the stereociliary bundles of vestibular hair cells are misoriented relative to their neighbors, a phenotype that is greatest in the cristae of the semicircular canals. Since horizontal semi-circular canal defects lead to circling in other mutant mouse lines, we propose that this PCP phenotype is the cellular basis of the circling behavior in Celsr1 mutants.</p>

Alternate JournalDev Biol
PubMed ID28159525
PubMed Central IDPMC5369242
Grant ListF32 DC014390 / DC / NIDCD NIH HHS / United States
P30 EY014800 / EY / NEI NIH HHS / United States
R01 DC013066 / DC / NIDCD NIH HHS / United States