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

Dev Biol. 2017 Mar 15;423(2):126-137. doi: 10.1016/j.ydbio.2017.01.020. Epub 2017 Feb 1.


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.

Publication types

  • Research Support, N.I.H., Extramural

MeSH terms

  • Animals
  • Behavior, Animal
  • Cell Polarity*
  • Ear, Inner / cytology*
  • Ear, Inner / embryology*
  • Ear, Inner / metabolism
  • Epithelium / metabolism
  • Gene Deletion
  • Hair Cells, Vestibular / cytology*
  • Mice, Knockout
  • Organ of Corti / cytology
  • Organ of Corti / embryology
  • Organ of Corti / metabolism
  • Phenotype
  • Receptors, G-Protein-Coupled / metabolism*
  • Signal Transduction
  • Stereocilia / metabolism


  • Celsr1 protein, mouse
  • Receptors, G-Protein-Coupled