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

Lukashkina, Victoria and Levic, Snezana and Lukashkin, Andrei and Strenzke, N (2021) A connexin30 mutation rescues hearing and reveals roles for gap junctions in cochlear amplification and micromechanics. [Data Collection]

Project Description

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 D-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.

Uncontrolled Keywords: mouse, cochlea, connexin 30, gap junctions, cochlear amplification
Subjects: B Subjects allied to medicine > B140 Neuroscience
Departments: School of Applied Sciences
Depositing User: Ian Russell
Date Deposited: 25 Feb 2021 16:46
Last Modified: 23 Jun 2021 11:39
Researchers (inc. External):
    • Lukashkina, Victoria
    • V.Lukashkina@brighton.ac.uk
    • Levic, Snezana
    • S.Levic@brighton.ac.uk
    • Lukashkin, Andrei
    • A.Lukashkin@brighton.ac.uk
    • Strenzke, N
    • NStrenzke@med.uni-goettingen.de
Temporal coverage:
1 September 201531 August 2020


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