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

Lukashkina, Victoria A, Levic, Snezana, Lukashkin, Andrei N, Strenzke, Nicola and Russell, Ian J (2017) A connexin30 mutation rescues hearing and reveals roles for gap junctions in cochlear amplification and micromechanics. Nature Communications, 8. a14530. ISSN 2041-1723

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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.

Item Type: Article
Keywords: Cellular motility; Cochlea; Intracellular recording; Neurophysiology
Schools and Departments: Brighton and Sussex Medical School > Neuroscience
Subjects: Q Science > QP Physiology > QP0351 Neurophysiology and neuropsychology
R Medicine > RF Otorhinolaryngology > RF0110 Otology. Diseases of the ear
Depositing User: Alexei Fisk
Date Deposited: 22 Jun 2017 11:21
Last Modified: 02 Jul 2019 17:33
URI: http://sro.sussex.ac.uk/id/eprint/68759

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