2017
Novel Role of the Mitochondrial Protein Fus1 in Protection from Premature Hearing Loss via Regulation of Oxidative Stress and Nutrient and Energy Sensing Pathways in the Inner Ear
Tan WJ, Song L, Graham M, Schettino A, Navaratnam D, Yarbrough WG, Santos-Sacchi J, Ivanova AV. Novel Role of the Mitochondrial Protein Fus1 in Protection from Premature Hearing Loss via Regulation of Oxidative Stress and Nutrient and Energy Sensing Pathways in the Inner Ear. Antioxidants & Redox Signaling 2017, 27: 489-509. PMID: 28135838, PMCID: PMC5564041, DOI: 10.1089/ars.2016.6851.Peer-Reviewed Original ResearchMeSH KeywordsAcetylcysteineAnimalsAntioxidantsDisease Models, AnimalEar, InnerEvoked Potentials, Auditory, Brain StemGene Knockout TechniquesHearing LossHumansMiceMicroscopy, Electron, TransmissionMitochondriaOxidative StressProto-Oncogene Proteins c-aktPTEN PhosphohydrolaseSignal TransductionSpiral GanglionTOR Serine-Threonine KinasesTumor Suppressor ProteinsConceptsAge-related hearing lossAuditory brainstem responseHearing lossKO miceEndocochlear potentialOxidative stressMitochondrial dysfunctionMitochondrial dysfunction/oxidative stressEnergy sensing pathwaysNovel therapeutic strategiesMolecular mechanismsPremature hearing lossCochlear stria vascularisMajor hearing lossChronic mitochondrial dysfunctionMetabolic etiologyWorldwide epidemicBrainstem responseClinical trialsVascular pathologyTherapeutic strategiesPathological alterationsABR parametersAO treatmentStria vascularis
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
1993
Voltage-dependent ionic conductances of type I spiral ganglion cells from the guinea pig inner ear
Santos-Sacchi J. Voltage-dependent ionic conductances of type I spiral ganglion cells from the guinea pig inner ear. Journal Of Neuroscience 1993, 13: 3599-3611. PMID: 8393487, PMCID: PMC6576533, DOI: 10.1523/jneurosci.13-08-03599.1993.Peer-Reviewed Original ResearchConceptsType I spiral ganglion cellsSpiral ganglion cellsOutward K currentsGuinea pig inner earGanglion cellsK currentsSpiral ganglion cell somataTTX-sensitive Na currentsInner earGanglion cell somataSpontaneous spike activityWhole-cell voltage clampNa currentIntrinsic membrane propertiesInner hair cellsSingle-unit studiesSingle-unit activityHalf-maximal amplitudeAfferent innervationCochlear nucleusIntrinsic electrical propertiesVoltage-dependent ionic conductancesCell somataExtracellular ionic environmentSpike activity