2019
Hearing consequences in Gjb2 knock-in mice: implications for human p.V37I mutation
Lin X, Li G, Zhang Y, Zhao J, Lu J, Gao Y, Liu H, Li G, Yang T, Song L, Wu H. Hearing consequences in Gjb2 knock-in mice: implications for human p.V37I mutation. Aging 2019, 11: 7416-7441. PMID: 31562289, PMCID: PMC6782001, DOI: 10.18632/aging.102246.Peer-Reviewed Original ResearchConceptsHomozygous miceLate-onset progressive hearing lossV37I mutationABR wave I latencySpiral ganglion neuron lossOuter hair cell functionSignificant hair cell lossMiddle ear injectionStria vascularis atrophyWave I latencyABR threshold elevationsProgressive hearing lossHair cell lossHair cell functionEnvironmental insultsI latencyNeuron lossCochlear pathologyHearing lossEar injectionMouse modelCell lossNoise exposureSystemic applicationThreshold elevation
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