2023
Role of mitochondrial dysfunction and oxidative stress in sensorineural hearing loss
Tan W, Song L. Role of mitochondrial dysfunction and oxidative stress in sensorineural hearing loss. Hearing Research 2023, 434: 108783. PMID: 37167889, DOI: 10.1016/j.heares.2023.108783.Peer-Reviewed Original ResearchConceptsSensorineural hearing lossOtotoxic drugsMitochondrial dysfunctionReactive oxygen speciesOxidative stressHearing lossDevelopment of SNHLMitochondrial reactive oxygen speciesSpiral ganglion neuronsMitochondrial DNA mutationsPrecise pathophysiological mechanismsSensory hair cellsHair cell mitochondriaConsequent oxidative stressCentral etiological roleMtDNA mutationsPathophysiological mechanismsGanglion neuronsAntioxidant therapyCalcium dysregulationDNA mutationsCell mitochondriaClinical studiesCochlear cellsEtiological role
2022
Outer hair cell function is normal in βV spectrin knockout mice
Stankewich MC, Bai JP, Stabach PR, Khan S, Tan WJT, Surguchev A, Song L, Morrow JS, Santos-Sacchi J, Navaratnam DS. Outer hair cell function is normal in βV spectrin knockout mice. Hearing Research 2022, 423: 108564. PMID: 35864018, DOI: 10.1016/j.heares.2022.108564.Peer-Reviewed Original ResearchConceptsOuter hair cellsAuditory brainstem response wavesAuditory thresholdOuter hair cell functionSpiral ganglion neuronsEfferent nerve fibersHair cell functionNumber of afferentsGanglion neuronsNerve fibersKnockout miceNeuronal structuresMiceHair cellsCell functionElectromechanical activityPutative roleType IOngoing investigationExon deletionsSynaptopathyAfferentsData supportResponse wavesNeurons
2020
Down-regulation of AMPK signaling pathway rescues hearing loss in TFB1 transgenic mice and delays age-related hearing loss
Zhao J, Li G, Zhao X, Lin X, Gao Y, Raimundo N, Li G, Shang W, Wu H, Song L. Down-regulation of AMPK signaling pathway rescues hearing loss in TFB1 transgenic mice and delays age-related hearing loss. Aging 2020, 12: 5590-5611. PMID: 32240104, PMCID: PMC7185105, DOI: 10.18632/aging.102977.Peer-Reviewed Original ResearchConceptsReactive oxygen speciesPro-survival functionRole of AMPKRegulation of AMPKActivation of AMPKAnti-apoptotic signalingPro-apoptotic signalingProtein kinaseOuter hair cellsAMPK activationAMPK hyperactivationROS-AMPKSpiral ganglion neuronsAMPK downregulationApoptosis pathwayHair cell synapsesAMPKMitochondrial dysfunctionCochlear tissuesCell growthRedox imbalanceBcl2 pathwayInner hair cell synapsesOxygen speciesHair cells
2019
Cellular Differences in the Cochlea of CBA and B6 Mice May Underlie Their Difference in Susceptibility to Hearing Loss
Liu H, Li G, Lu J, Gao Y, Song L, Li G, Wu H. Cellular Differences in the Cochlea of CBA and B6 Mice May Underlie Their Difference in Susceptibility to Hearing Loss. Frontiers In Cellular Neuroscience 2019, 13: 60. PMID: 30873008, PMCID: PMC6400987, DOI: 10.3389/fncel.2019.00060.Peer-Reviewed Original ResearchInner hair cellsEarly-onset hearing lossB6 miceOnset hearing lossHearing lossRibbon synapsesOuter hair cell functionMouse strainsCBA/CaJ miceWave I latencySpiral ganglion neuronsSynaptic vesiclesHair cell functionCellular differencesStable hearingSimilar hearing thresholdsI latencyABR thresholdGanglion neuronsGlutamate toxicityHearing thresholdsWave IMiceHair cellsCell function
2018
Characterizing a novel vGlut3-P2A-iCreER knockin mouse strain in cochlea
Li C, Shu Y, Wang G, Zhang H, Lu Y, Li X, Li G, Song L, Liu Z. Characterizing a novel vGlut3-P2A-iCreER knockin mouse strain in cochlea. Hearing Research 2018, 364: 12-24. PMID: 29706463, DOI: 10.1016/j.heares.2018.04.006.Peer-Reviewed Original ResearchMeSH KeywordsAcoustic StimulationAmino Acid Transport Systems, AcidicAnimalsCochleaEvoked Potentials, Auditory, Brain StemFemaleGene Knock-In TechniquesGenes, ReporterGenotypeHair Cells, Auditory, OuterIntegrasesLuminescent ProteinsMaleMice, Inbred C57BLMice, TransgenicNeurogliaPhenotypeReaction TimeReceptors, EstrogenSelective Estrogen Receptor ModulatorsSpiral GanglionTamoxifenTime FactorsConceptsInner hair cellsOuter hair cellsSpiral ganglion neuronsKnockin mouse strainGlia cellsVesicular glutamate transporter 3Mouse strainsHair cellsCochlear outer hair cellsRosa26-LSLVGLUT3 expressionGanglion neuronsVGLUT3Mouse cochleaTransporter 3Negative cellsMouse genetic studiesAntibody stainingTamoxifenUnique expression patternP2/P3Specific cell typesCell typesSitu hybridizationCochlea
2015
Auditory Pathology in a Transgenic mtTFB1 Mouse Model of Mitochondrial Deafness
McKay SE, Yan W, Nouws J, Thormann MJ, Raimundo N, Khan A, Santos-Sacchi J, Song L, Shadel GS. Auditory Pathology in a Transgenic mtTFB1 Mouse Model of Mitochondrial Deafness. American Journal Of Pathology 2015, 185: 3132-3140. PMID: 26552864, PMCID: PMC5801480, DOI: 10.1016/j.ajpath.2015.08.014.Peer-Reviewed Original ResearchMeSH KeywordsAMP-Activated Protein KinasesAnimalsApoptosisDeafnessDisease Models, AnimalDNA, MitochondrialEvoked Potentials, Auditory, Brain StemHair Cells, Auditory, InnerMice, Inbred C57BLMice, KnockoutMice, TransgenicMitochondrial DiseasesMutationOrgan of CortiReaction TimeSignal TransductionSpiral GanglionStria VascularisTranscription FactorsConceptsAMP kinaseReactive oxygen species-mediated activationTranscription factor E2F1A1555G mutationAuditory pathologyHair cellsTFB1MHearing loss phenotypeRRNA geneAMPK-α1AMPK activityProlonged wave I latencyLoss phenotypeMitochondrial pathologyNonsyndromic deafnessTransgenic mouse strainWave I latencySpiral ganglion neuronsProgressive hearing lossMitochondrial deafnessPotential therapeutic valueDNA causeG mutationOuter hair cellsI latency