1. Genetics of auditory mechano-electrical transduction
- Author
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Nicolas Michalski, Christine Petit, Chaire Génétique et physiologie cellulaire, Collège de France (CdF (institution)), Institut de la Vision, Centre National de la Recherche Scientifique (CNRS)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Université Pierre et Marie Curie - Paris 6 (UPMC), The authors’ research is supported by grants from the European Research council (ERC-2011-AdG 294570 HAIRBUNDLE), the Agence nationale pour la recherche (EARMEC—ANR 11 BSV5 011 02), the LABEX LIFESENSES (reference ANR-10-LABX-65) supported by French state funds within the Investissementsd’Avenir programme under reference ANR-11-IDEX-0004-02, BNPParibas and LHW Stiftung, ANR-11-BSV5-0011,EARMEC,Propriétés mécaniques, actives et passives, de la touffe ciliaire des cellules mécano-sensorielles ciliées le long de l'axe tonotopique de la cochlée des mammifères.(2011), ANR-11-IDEX-0004,SUPER,Sorbonne Universités à Paris pour l'Enseignement et la Recherche(2011), European Project: 294570,EC:FP7:ERC,ERC-2011-ADG_20110310,HAIRBUNDLE(2012), Collège de France - Chaire Génétique et physiologie cellulaire, Université Pierre et Marie Curie - Paris 6 (UPMC)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS), michalski, nicolas, BLANC - Propriétés mécaniques, actives et passives, de la touffe ciliaire des cellules mécano-sensorielles ciliées le long de l'axe tonotopique de la cochlée des mammifères. - - EARMEC2011 - ANR-11-BSV5-0011 - BLANC - VALID, Sorbonne Universités à Paris pour l'Enseignement et la Recherche - - SUPER2011 - ANR-11-IDEX-0004 - IDEX - VALID, and Assembling the puzzle of the operating auditory hair bundle - HAIRBUNDLE - - EC:FP7:ERC2012-12-01 - 2017-11-30 - 294570 - VALID
- Subjects
Physiology ,[SDV.NEU.NB]Life Sciences [q-bio]/Neurons and Cognition [q-bio.NC]/Neurobiology ,Clinical Biochemistry ,MESH: Molecular Motor Proteins ,Action Potentials ,Mechanotransduction, Cellular ,Membrane Potentials ,0302 clinical medicine ,Hearing ,MESH: Hair Cells, Auditory ,MESH: Animals ,Neurogenetics ,MESH: Action Potentials ,Genetics ,0303 health sciences ,Molecular Motor Proteins ,Hair bundle ,Cochlea ,Molecular network ,medicine.anatomical_structure ,[SDV.MHEP.OS] Life Sciences [q-bio]/Human health and pathology/Sensory Organs ,Hair cell ,MESH: Membrane Proteins ,MESH: Pressure ,Transduction (physiology) ,Mechano-electrical transduction ,Biology ,03 medical and health sciences ,Physiology (medical) ,Hair Cells, Auditory ,Pressure ,medicine ,otorhinolaryngologic diseases ,Animals ,Humans ,MESH: Membrane Potentials ,[SDV.MHEP.OS]Life Sciences [q-bio]/Human health and pathology/Sensory Organs ,MESH: Hearing ,030304 developmental biology ,Invited Review ,MESH: Humans ,MESH: Mechanotransduction, Cellular ,[SDV.NEU.NB] Life Sciences [q-bio]/Neurons and Cognition [q-bio.NC]/Neurobiology ,Membrane Proteins ,Human physiology ,Mechano electrical transduction ,sense organs ,Neuroscience ,030217 neurology & neurosurgery - Abstract
International audience; The hair bundles of cochlear hair cells play a central role in the auditory mechano-electrical transduction (MET) process. The identification of MET components and of associated molecular complexes by biochemical approaches is impeded by the very small number of hair cells within the cochlea. In contrast, human and mouse genetics have proven to be particularly powerful. The study of inherited forms of deafness led to the discovery of several essential proteins of the MET machinery, which are currently used as entry points to decipher the associated molecular networks. Notably, MET relies not only on the MET machinery but also on several elements ensuring the proper sound-induced oscillation of the hair bundle or the ionic environment necessary to drive the MET current. Here, we review the most significant advances in the molecular bases of the MET process that emerged from the genetics of hearing.
- Published
- 2015
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