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 mutationsMiceProgressive age-dependence and frequency difference in the effect of gap junctions on active cochlear amplification and hearing
Zong L, Chen J, Zhu Y, Zhao H. Progressive age-dependence and frequency difference in the effect of gap junctions on active cochlear amplification and hearing. Biochemical And Biophysical Research Communications 2017, 489: 223-227. PMID: 28552523, PMCID: PMC5555358, DOI: 10.1016/j.bbrc.2017.05.137.Peer-Reviewed Original ResearchMeSH KeywordsAgingAnimalsCochleaConnexin 26ConnexinsGap JunctionsHearing LossMiceMice, KnockoutMice, TransgenicConceptsActive cochlear amplificationHearing lossCochlear amplificationMice ageGap junctionsAge-related hearing lossSignificant hearing lossPostnatal day 25Cochlear gap junctionsAuditory sensory hair cellsSensory hair cellsNonsyndromic hearing lossHigh incidenceOuter pillar cellsDay 25Deiters' cellsConnexin expressionHair cellsConnexin 26Outer hair cell electromotilityHair cell electromotilityPillar cellsPrevious reportsCochleaAge
2015
Connexin26 gap junction mediates miRNA intercellular genetic communication in the cochlea and is required for inner ear development
Zhu Y, Zong L, Mei L, Zhao H. Connexin26 gap junction mediates miRNA intercellular genetic communication in the cochlea and is required for inner ear development. Scientific Reports 2015, 5: 15647. PMID: 26490746, PMCID: PMC4614881, DOI: 10.1038/srep15647.Peer-Reviewed Original ResearchConceptsGenetic communicationOrgan developmentInner ear gap junctionsIntercellular communicationGap junctionsCochlear developmentInner ear developmentNon-coding RNAsCx26 knockout miceEar developmentGene expressionIntercellular transferCx26 deficiencyMiR-96 expressionCx30 deficiencyDevelopmental disordersPredominant isoformCell proliferationDeletionCx26Critical roleKnockout miceExpressionMiRNAsMicroRNAs
2014
Connexin26 (GJB2) deficiency reduces active cochlear amplification leading to late-onset hearing loss
Zhu Y, Chen J, Liang C, Zong L, Chen J, Jones R, Zhao H. Connexin26 (GJB2) deficiency reduces active cochlear amplification leading to late-onset hearing loss. Neuroscience 2014, 284: 719-729. PMID: 25451287, PMCID: PMC4268423, DOI: 10.1016/j.neuroscience.2014.10.061.Peer-Reviewed Original ResearchConceptsLate-onset hearing lossActive cochlear amplificationDistortion product otoacoustic emissionsHearing lossNonsyndromic hearing lossTherapeutic interventionsProgressive hearing lossHair cell lossPostnatal day 5Cochlear amplificationProduct otoacoustic emissionsConditional knockout miceKnockout miceClinical observationsDay 5Cell lossEndocochlear potentialOtoacoustic emissionsNormal hearingCx26 expressionDeafness mechanismMiceCx26 deficiencyCochleaInterventionDeafness induced by Connexin 26 (GJB2) deficiency is not determined by endocochlear potential (EP) reduction but is associated with cochlear developmental disorders
Chen J, Chen J, Zhu Y, Liang C, Zhao H. Deafness induced by Connexin 26 (GJB2) deficiency is not determined by endocochlear potential (EP) reduction but is associated with cochlear developmental disorders. Biochemical And Biophysical Research Communications 2014, 448: 28-32. PMID: 24732355, PMCID: PMC4105360, DOI: 10.1016/j.bbrc.2014.04.016.Peer-Reviewed Original ResearchConceptsAuditory brainstem responseHair cell degenerationKO miceCongenital deafnessEP reductionEndocochlear potentialHearing lossCell degenerationDevelopmental disordersActive cochlear amplificationCx26 knockout miceComplete hearing lossCx26 deficiencyPostnatal day 5Connexin 26 mutationsNonsyndromic hearing lossBrainstem responseMouse modelKnockout miceDay 5Deafness mechanismMajor causeMiceDeafnessDisorders
2013
Active cochlear amplification is dependent on supporting cell gap junctions
Zhu Y, Liang C, Chen J, Zong L, Chen G, Zhao H. Active cochlear amplification is dependent on supporting cell gap junctions. Nature Communications 2013, 4: 1786. PMID: 23653198, PMCID: PMC3675877, DOI: 10.1038/ncomms2806.Peer-Reviewed Original ResearchMeSH KeywordsAnimalsAuditory ThresholdCochlear Microphonic PotentialsConnexin 26ConnexinsEvoked Potentials, Auditory, Brain StemGap JunctionsGene DeletionGene TargetingHair Cells, Auditory, OuterHearing LossLabyrinth Supporting CellsMiceMice, KnockoutMolecular Motor ProteinsNonlinear DynamicsOtoacoustic Emissions, SpontaneousSpiral GanglionConceptsActive cochlear amplificationOuter hair cellsCell gap junctionsHearing lossCochlear amplificationHair cellsGap junctionsDistortion product otoacoustic emissionsOuter hair cell electromotilityHair cell electromotilitySevere hearing lossProduct otoacoustic emissionsShorter outer hair cellsHair-bundle movementsOuter pillar cellsLeftward shiftOtoacoustic emissionsAcoustic stimulationDeiters' cellsHearing sensitivityConnexin 26Active cochlear mechanicsNovel findingsPillar cellsBundle movement
2012
Cell degeneration is not a primary causer for Connexin26 (GJB2) deficiency associated hearing loss
Liang C, Zhu Y, Zong L, Lu G, Zhao H. Cell degeneration is not a primary causer for Connexin26 (GJB2) deficiency associated hearing loss. Neuroscience Letters 2012, 528: 36-41. PMID: 22975134, PMCID: PMC3467974, DOI: 10.1016/j.neulet.2012.08.085.Peer-Reviewed Original ResearchConceptsHair cell lossAuditory brainstem responseCell degenerationCell lossNeuron degenerationPostnatal developmentCx26 deficiencyCochlear hair cell lossSpiral ganglion neuron degenerationDevelopment disordersCx26 knockout miceHair cellsHair cell functionOuter hair cellsSG neuronsNonsyndromic hearing lossKO miceBrainstem responseCochlear cellsHearing lossBasal turnMouse modelKnockout miceCongenital deafnessSignificant degeneration
2008
Cellular characterization of Connexin26 and Connnexin30 expression in the cochlear lateral wall
Liu Y, Zhao H. Cellular characterization of Connexin26 and Connnexin30 expression in the cochlear lateral wall. Cell And Tissue Research 2008, 333: 395. PMID: 18581144, PMCID: PMC2548271, DOI: 10.1007/s00441-008-0641-5.Peer-Reviewed Original Research
2006
Distinct and gradient distributions of connexin26 and connexin30 in the cochlear sensory epithelium of guinea pigs
Zhao H, Yu N. Distinct and gradient distributions of connexin26 and connexin30 in the cochlear sensory epithelium of guinea pigs. The Journal Of Comparative Neurology 2006, 499: 506-518. PMID: 16998915, PMCID: PMC2553046, DOI: 10.1002/cne.21113.Peer-Reviewed Original ResearchConceptsCochlear sensory epitheliumSensory epitheliumGuinea pigsHensen's cellsDeiters' cellsSpiral ganglion neuronsPillar cellsExpression of Cx26Auditory sensory epitheliumDistinct cellular expressionGanglion neuronsCochlear apexCx26 labelingCell bodiesCx26 expressionImmunofluorescent stainingEpitheliumHair cellsCellular expressionCx30Dense labelingClaudius cellsCell preparationsPredominant isoformCellular distribution
2005
Connexin26 is responsible for anionic molecule permeability in the cochlea for intercellular signalling and metabolic communications
Zhao H. Connexin26 is responsible for anionic molecule permeability in the cochlea for intercellular signalling and metabolic communications. European Journal Of Neuroscience 2005, 21: 1859-1868. PMID: 15869481, PMCID: PMC2548270, DOI: 10.1111/j.1460-9568.2005.04031.x.Peer-Reviewed Original ResearchMeSH KeywordsAdenosine TriphosphateAffinity LabelsAnimalsAnionsAnti-Inflammatory AgentsCalciumCationsCell CommunicationCell CountCell Membrane PermeabilityCells, CulturedCochleaConnexin 26ConnexinsDose-Response Relationship, DrugEnzyme InhibitorsFluorescent DyesGap JunctionsGlycyrrhetinic AcidGuinea PigsIntracellular MembranesPlatelet Aggregation InhibitorsProadifenPyridoxal PhosphateSpectrometry, FluorescenceTime FactorsConceptsCharge selectivityLarge pore sizeCationic fluorescent dyeIntercellular signalingAnionic dyesDye sizeGap junctionsMetabolic communicationPore sizeMolecule permeabilityMolecular permeabilitySelectivityEnergy moleculesCationic probePassage of ionsFluorescent dyeGap junctional permeabilityMoleculesCochlear sensory epitheliumDyeCochlear gap junctions