%0 Book Section %T Cadherins in the Auditory Sensory Organ %+ Génétique et Physiologie de l'Audition %A El-Amraoui, Aziz %A Petit, Christine %Z The work of the authors is supported by Institut Pasteur, INSERM, the European Union Seventh Framework Programme, under grant agreement HEALTH-F2-2010-242013 (TREATRUSH), LHW-Stiftung, Fondation Raymonde & Guy Strittmatter, Fighting Blindness, FAUN Stiftung (Suchert Foundation), Conny Maeva Charitable Foundation, Fondation Orange, European Research Council (ERC) advanced grant “Hair bundle” (ERC-2011-AdG 294570), LABEX Lifesenses [ANR-10-LABX-65], the French National Research Agency (ANR) as part of the second “Investissements d’Avenir” programme (ANR-15-RHUS-0001), Retina France, and the Fondation Voir et Entendre. %@ 978-4-431-56033-3 %B The Cadherin Superfamily %E Suzuki, S %E Hirano, S %I Springer %C Tokyo %P 341 - 361 %8 2016-06-03 %D 2016 %R 10.1007/978-4-431-56033-3_13 %K Cadherins Usher syndrome Deafness Convergent extension Planar cell polarity Cell-cell contacts Tip-link Stereocilia %K Cadherins %K Usher syndrome %K Deafness %K Convergent extension %K Planar cell polarity %K Cell-cell contacts %K Tip-link %K Stereocilia %Z Life Sciences [q-bio]Book sections %X The exquisite sensitivity and frequency tuning of hearing depend on the correct structure and functioning of the auditory sensory hair cells, the neighbouring supporting cells, and the homeostasis of their ionic environment. The increasing number of adhesion proteins identified as causing hearing impairment in humans and mice when defective is consistent with a critical role for cellcell junctions between neighbouring epithelial cells of the cochlea, and of fibrous links within the hair bundle, the sensory hair cell structure responsible for sound reception. Classical cadherins and/or associated adherens-junction proteins, such as p120-catenin or nectin 3, have been shown to be essential for establishment of the regular mosaic cellular pattern of the auditory sensory epithelium. Two cadherinrelated proteins, protocadherin-15 and cadherin-23, are key components of both lateral links and tip-links in hair bundles; they are essential components of the mechanoelectrical transduction machinery. Studies of the role of these adhesion proteins and of the pathogenesis of the forms of deafness caused by defects of these proteins have provided considerable insight into the development and functioning of the auditory sensory epithelium, and of the hair cells in particular. %G English %L pasteur-03922347 %U https://pasteur.hal.science/pasteur-03922347 %~ PASTEUR %~ UPMC %~ OPENAIRE %~ U1120 %~ UPMC_POLE_4 %~ SORBONNE-UNIVERSITE %~ SU-MEDECINE %~ SU-MED %~ ANR %~ ALLIANCE-SU %~ INSTITUT-AUDITION