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A connexin30 mutation rescues hearing and reveals roles for gap junctions in cochlear amplification and micromechanics

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  • Victoria A. Lukashkina

    (Sensory Neuroscience Research Group, School of Pharmacy and Biomolecular Sciences, University of Brighton)

  • Snezana Levic

    (Sensory Neuroscience Research Group, School of Pharmacy and Biomolecular Sciences, University of Brighton
    Brighton and Sussex Medical School, University of Sussex)

  • Andrei N. Lukashkin

    (Sensory Neuroscience Research Group, School of Pharmacy and Biomolecular Sciences, University of Brighton)

  • Nicola Strenzke

    (University Medicine Göttingen)

  • Ian J. Russell

    (Sensory Neuroscience Research Group, School of Pharmacy and Biomolecular Sciences, University of Brighton)

Abstract

Accelerated age-related hearing loss disrupts high-frequency hearing in inbred CD-1 mice. The p.Ala88Val (A88V) mutation in the gene coding for the gap-junction protein connexin30 (Cx30) protects the cochlear basal turn of adult CD-1Cx30A88V/A88V mice from degeneration and rescues hearing. Here we report that the passive compliance of the cochlear partition and active frequency tuning of the basilar membrane are enhanced in the cochleae of CD-1Cx30A88V/A88V compared to CBA/J mice with sensitive high-frequency hearing, suggesting that gap junctions contribute to passive cochlear mechanics and energy distribution in the active cochlea. Surprisingly, the endocochlear potential that drives mechanoelectrical transduction currents in outer hair cells and hence cochlear amplification is greatly reduced in CD-1Cx30A88V/A88V mice. Yet, the saturating amplitudes of cochlear microphonic potentials in CD-1Cx30A88V/A88V and CBA/J mice are comparable. Although not conclusive, these results are compatible with the proposal that transmembrane potentials, determined mainly by extracellular potentials, drive somatic electromotility of outer hair cells.

Suggested Citation

  • Victoria A. Lukashkina & Snezana Levic & Andrei N. Lukashkin & Nicola Strenzke & Ian J. Russell, 2017. "A connexin30 mutation rescues hearing and reveals roles for gap junctions in cochlear amplification and micromechanics," Nature Communications, Nature, vol. 8(1), pages 1-10, April.
    • Handle: RePEc:nat:natcom:v:8:y:2017:i:1:d:10.1038_ncomms14530
    DOI: 10.1038/ncomms14530
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