2022
In vivo CRISPR‐Cas9‐mediated DNA chop identifies a cochlear outer hair cell‐specific enhancer
Sun Y, Zhang Y, Zhang D, Wang G, Song L, Liu Z. In vivo CRISPR‐Cas9‐mediated DNA chop identifies a cochlear outer hair cell‐specific enhancer. The FASEB Journal 2022, 36: e22233. PMID: 35225354, DOI: 10.1096/fj.202100421rr.Peer-Reviewed Original ResearchConceptsDNA fragment deletionKbp fragmentKbp segmentFragment deletionCell-specific enhancerOuter hair cellsLarge DNA fragment deletionGreen fluorescent proteinCRISPR/Motor proteinsIntron regionsGene therapeutic applicationsFluorescent proteinDifferent speciesCochlear outer hair cellsBp fragmentEnhancerSLC26A5Hair cellsEGFPProteinTransgenic miceDeletionKbpPrestin expression
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
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
2012
Mitochondrial Stress Engages E2F1 Apoptotic Signaling to Cause Deafness
Raimundo N, Song L, Shutt TE, McKay SE, Cotney J, Guan MX, Gilliland TC, Hohuan D, Santos-Sacchi J, Shadel GS. Mitochondrial Stress Engages E2F1 Apoptotic Signaling to Cause Deafness. Cell 2012, 148: 716-726. PMID: 22341444, PMCID: PMC3285425, DOI: 10.1016/j.cell.2011.12.027.Peer-Reviewed Original ResearchConceptsAltered reactive oxygen speciesReactive oxygen speciesMitochondrial ribosome functionMitochondrial disease modelTranscription factor E2F1Tissue-specific pathologyROS-dependent activationRibosome functionRRNA methylationMitochondrial stressApoptotic signalingTissue specificityMtDNA mutationsMetabolic signalingAMP kinaseMultiple tissuesMitochondrial dysfunctionOxygen speciesE2F1MethylationSignalingG cellsEnvironmental factorsApoptosisMice exhibit