2023
Cx26 heterozygous mutations cause hyperacusis-like hearing oversensitivity and increase susceptibility to noise
Liu L, Liang C, Chen J, Fang S, Zhao H. Cx26 heterozygous mutations cause hyperacusis-like hearing oversensitivity and increase susceptibility to noise. Science Advances 2023, 9: eadf4144. PMID: 36753545, PMCID: PMC9908021, DOI: 10.1126/sciadv.adf4144.Peer-Reviewed Original ResearchConceptsActive cochlear amplificationCochlear amplificationHeterozygous mutationsPermanent hearing threshold shiftHearing threshold shiftCochlear lateral wallNonsyndromic hearing lossHearing lossMouse modelGeneral populationNoise exposureThreshold shiftHeterozygote carriersHearing sensitivityLateral wallJunction genesGap junction genesPotential generationUnexpected findingExposureMutationsOversensitivityProtein prestinDeafness
2017
A deafness mechanism of digenic Cx26 (GJB2) and Cx30 (GJB6) mutations: Reduction of endocochlear potential by impairment of heterogeneous gap junctional function in the cochlear lateral wall
Mei L, Chen J, Zong L, Zhu Y, Liang C, Jones R, Zhao H. A deafness mechanism of digenic Cx26 (GJB2) and Cx30 (GJB6) mutations: Reduction of endocochlear potential by impairment of heterogeneous gap junctional function in the cochlear lateral wall. Neurobiology Of Disease 2017, 108: 195-203. PMID: 28823936, PMCID: PMC5675824, DOI: 10.1016/j.nbd.2017.08.002.Peer-Reviewed Original ResearchConceptsCochlear lateral wallEndocochlear potentialHearing lossGap junctional functionDeafness mechanismLateral wallHeterozygous miceCx30 mutationsHair cell degenerationHomozygous knockout miceJunctional functionHeterozygous mouse modelGap junctionsOrgan of CortiSame gap junctional plaquesEP reductionFrequent causePathological changesMouse modelKnockout miceReceptor currentsCell degenerationNormal hearingHeterozygous mutationsMice
2016
Expression and function of pannexins in the inner ear and hearing
Zhao H. Expression and function of pannexins in the inner ear and hearing. BMC Molecular And Cell Biology 2016, 17: 16. PMID: 27229462, PMCID: PMC4896268, DOI: 10.1186/s12860-016-0095-7.Peer-Reviewed Original ResearchConceptsFunction of pannexinsAuditory sensory hair cellsDistinct expression patternsCell apoptotic pathwaysGap junction genesSensory hair cellsGap junction proteinGene familyATP releaseExpression patternsApoptotic pathwayEndocochlear potentialJunction genesPannexinsActive cochlear amplificationPannexin expressionLateral wallCochlear lateral wallJunction proteinsOrgan of CortiHair cellsCritical roleCochlear amplificationIsoformsStria vascularis
2015
Pannexin1 channels dominate ATP release in the cochlea ensuring endocochlear potential and auditory receptor potential generation and hearing
Chen J, Zhu Y, Liang C, Chen J, Zhao H. Pannexin1 channels dominate ATP release in the cochlea ensuring endocochlear potential and auditory receptor potential generation and hearing. Scientific Reports 2015, 5: 10762. PMID: 26035172, PMCID: PMC4451810, DOI: 10.1038/srep10762.Peer-Reviewed Original ResearchConceptsCochlear lateral wallATP releaseHearing lossCochlear microphonicsPotential generationReceptor potentialReceptor potential generationHair cell lossLateral wallNon-junctional channelsEP generationDeficient miceCell lossEndocochlear potentialHair cellsPathological processesCochleaPhysiological conditionsJunction genesGap junction genesConnexin hemichannelsConnexin isoformsHearingDeficiencyRelease
2008
Identification and characterization of pannexin expression in the mammalian cochlea
Wang X, Streeter M, Liu Y, Zhao H. Identification and characterization of pannexin expression in the mammalian cochlea. The Journal Of Comparative Neurology 2008, 512: 336-346. PMID: 19009624, PMCID: PMC2630187, DOI: 10.1002/cne.21898.Peer-Reviewed Original ResearchConceptsSpiral ganglion neuronsCochlear lateral wallDiffuse cytoplasmic labelingMammalian cochleaType II fibrocytesOrgan of CortiBlood vessel cellsOuter sulcus cellsWestern blot analysisDistinct cellular distributionGanglion neuronsPolymerase chain reactionGap junctional proteinRat cochleaInterdental cellsStria vascularisPunctate labelingDeiters' cellsSpiral limbusImmunofluorescent stainingCochlear boneHair cellsBasal cellsSpiral prominencePannexin expression