title: Planar cell polarity in the inner ear.
abstract: The inner ears of vertebrates represent one of the most striking examples of planar cell polarity (PCP). Populations of directionally sensitive mechanosensory hair cells develop actin-based stereociliary bundles that are uniformly oriented. Analysis of perturbations in bundle polarity in mice with mutations in Vangl2 formed the basis for the initial demonstration of conservation of the PCP signaling pathway in vertebrates. Subsequent studies have demonstrated roles for other "core" PCP genes, such as Frizzled, Disheveled, and Celsr, and for identifying novel PCP molecules such as Scribble and Ptk7. In addition, the demonstration of hearing deficits in humans with mutations in cilia genes combined with analysis of PCP defects in mice with ciliary deletion has implicated the cilia as an important modulator of hair cell polarization. Finally, the presence of shortened cochleae in many PCP mouse mutants has revealed an additional role for the PCP pathway in the development of the auditory system.
authors.affiliation: Laboratory of Cochlear Development, NIDCD, NIH, Bethesda, Maryland, USA.
authors.forename: Helen
authors.initials: H
authors.lastname: May-Simera
authors.forename: Matthew W
authors.initials: MW
authors.lastname: Kelley
mesh_terms: D008841:Actin Cytoskeleton / Q000235:genetics / Q000378:metabolism; D000818:Animals; D015820:Cadherins / Q000235:genetics / Q000378:metabolism; D016764:Cell Polarity; D002923:Cilia / Q000235:genetics / Q000378:metabolism; D003593:Cytoplasm / Q000235:genetics / Q000378:metabolism; D004330:Drosophila / Q000166:cytology / Q000378:metabolism; D029721:Drosophila Proteins / Q000235:genetics / Q000378:metabolism; D007758:Ear, Inner / Q000166:cytology* / Q000378:metabolism; D006198:Hair Cells, Auditory / Q000166:cytology / Q000378:metabolism*; D040542:Mechanotransduction, Cellular; D051379:Mice; D009154:Mutation; D009419:Nerve Tissue Proteins / Q000235:genetics / Q000378:metabolism; D009436:Neural Tube Defects / Q000378:metabolism / Q000473:pathology; D020794:Receptor Protein-Tyrosine Kinases / Q000235:genetics / Q000378:metabolism
chemical_list: D015820:Cadherins; D029721:Drosophila Proteins; C431577:Ltap protein, mouse; D009419:Nerve Tissue Proteins; C094449:ds protein, Drosophila; C486452:Ptk7 protein, mouse; D020794:Receptor Protein-Tyrosine Kinases