A connexin30 mutation rescues hearing and reveals roles for gap junctions in cochlear amplification and micromechanics.pdf (1.07 MB)
A connexin30 mutation rescues hearing and reveals roles for gap junctions in cochlear amplification and micromechanics
journal contribution
posted on 2023-06-09, 06:50 authored by Victoria A Lukashkina, Snezana LevicSnezana Levic, Andrei N Lukashkin, Nicola Strenzke, Ian J RussellAccelerated 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.
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Publication status
- Published
File Version
- Published version
Journal
Nature CommunicationsISSN
2041-1723Publisher
Nature Publishing GroupExternal DOI
Volume
8Article number
a14530Department affiliated with
- BSMS Neuroscience Publications
Full text available
- Yes
Peer reviewed?
- Yes
Legacy Posted Date
2017-06-22First Open Access (FOA) Date
2017-06-22First Compliant Deposit (FCD) Date
2017-06-22Usage metrics
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