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
1985
A Ferritin-containing Cell Type in the Stria Vascularis of the Mouse Inner Ear
Santos-Sacchi J, Marovitz W. A Ferritin-containing Cell Type in the Stria Vascularis of the Mouse Inner Ear. Acta Oto-Laryngologica 1985, 100: 26-32. PMID: 4024891, DOI: 10.3109/00016488509108583.Peer-Reviewed Original Research
1982
An electronmicroscopic study of microtubules in the development of marginal cells of the mouse stria vascularis
Santos-Sacchi J. An electronmicroscopic study of microtubules in the development of marginal cells of the mouse stria vascularis. Hearing Research 1982, 6: 7-13. PMID: 7054137, DOI: 10.1016/0378-5955(82)90003-x.Peer-Reviewed Original ResearchMeSH KeywordsAge FactorsAnimalsCochleaMiceMice, Inbred StrainsMicroscopy, ElectronMicrotubulesMitochondriaStria VascularisConceptsMouse stria vascularisPlasma membraneCellular extensionsNumerous cellular processesMarginal cellsSheet-like extensionsCellular processesCell peripheryLength of microtubulesGolgi bodiesDepolymerization of microtubulesCytoplasmic microtubulesMicrotubulesNumerous microtubulesCell processesOrganellesMitochondriaStria vascularisCellsMembraneAdult miceCytoplasmVesiclesPlasmalemmaMicrotubles
1980
An Evaluation of Normal Strial Capillary Transport Using the Electron-Opaque Tracers Ferritin and Iron Dextran
Santos-Sacchi J, Marovitz W. An Evaluation of Normal Strial Capillary Transport Using the Electron-Opaque Tracers Ferritin and Iron Dextran. Acta Oto-Laryngologica 1980, 89: 12-26. PMID: 7405570, DOI: 10.3109/00016488009127103.Peer-Reviewed Original Research
1978
Differential Effects of Primary Fixation with Glutaraldehyde and Osmium Upon the Membranous Systems of the Strial and External Sulcus Cells
Santos-Sacchi J. Differential Effects of Primary Fixation with Glutaraldehyde and Osmium Upon the Membranous Systems of the Strial and External Sulcus Cells. Acta Oto-Laryngologica 1978, 86: 56-63. PMID: 99973, DOI: 10.3109/00016487809124720.Peer-Reviewed Original Research