2015
Cellular and Deafness Mechanisms Underlying Connexin Mutation-Induced Hearing Loss – A Common Hereditary Deafness
Wingard J, Zhao H. Cellular and Deafness Mechanisms Underlying Connexin Mutation-Induced Hearing Loss – A Common Hereditary Deafness. Frontiers In Cellular Neuroscience 2015, 9: 202. PMID: 26074771, PMCID: PMC4448512, DOI: 10.3389/fncel.2015.00202.Peer-Reviewed Original ResearchHearing lossPathological changesDeafness mechanismCongenital deafnessActive cochlear amplificationProgressive hearing lossDetailed cellular mechanismsCochlear hair cellsHair cell degenerationHereditary deafnessConnexin 26 mutationsDistinct pathological changesNon-syndromic hearing lossProfound congenital deafnessAuditory phenotypeHigh incidenceTherapeutic strategiesGap junctional proteinCell degenerationConnexin expressionHair cellsDeafnessCellular mechanismsLate childhoodCx26 mutations
2004
A “Synaptoplasmic Cistern” Mediates Rapid Inhibition of Cochlear Hair Cells
Lioudyno M, Hiel H, Kong JH, Katz E, Waldman E, Parameshwaran-Iyer S, Glowatzki E, Fuchs PA. A “Synaptoplasmic Cistern” Mediates Rapid Inhibition of Cochlear Hair Cells. Journal Of Neuroscience 2004, 24: 11160-11164. PMID: 15590932, PMCID: PMC6730265, DOI: 10.1523/jneurosci.3674-04.2004.Peer-Reviewed Original ResearchConceptsCalcium-induced calcium releaseCochlear hair cellsHair cellsCholinergic efferent neuronsOuter hair cellsExogenous AChEfferent neuronsSynaptic releaseSynaptic inputsRat cochleaApical turnAcetylcholine receptorsCalcium releaseCalcium entersPotassium channelsCation channelsSarcoplasmic reticulumAChType IReceptorsRapid inhibitionCisternsCellsNeuronsRelease
1995
Expression in cochlea and retina of myosin VIIa, the gene product defective in Usher syndrome type 1B.
Hasson T, Heintzelman M, Santos-Sacchi J, Corey D, Mooseker M. Expression in cochlea and retina of myosin VIIa, the gene product defective in Usher syndrome type 1B. Proceedings Of The National Academy Of Sciences Of The United States Of America 1995, 92: 9815-9819. PMID: 7568224, PMCID: PMC40893, DOI: 10.1073/pnas.92.21.9815.Peer-Reviewed Original ResearchMeSH KeywordsAbnormalities, MultipleAmino Acid SequenceAnimalsAntibody SpecificityBlindnessCochleaDeafnessDyneinsFluorescent Antibody TechniqueGuinea PigsHearing Loss, SensorineuralHumansImmunoblottingMolecular Sequence DataMyosin VIIaMyosinsRatsRetinaRetinitis PigmentosaSequence Homology, Amino AcidSwineSyndromeTissue DistributionConceptsHair cellsMyosin VIIa expressionEpithelial cellsMyosin VIIaCochlear hair cellsMyosin VIIa functionOuter hair cellsUsher syndrome type 1BMyosin VIIA geneVestibular dysfunctionCell-specific localizationCongenital deafnessRetinitis pigmentosaUsher syndromeType 1bType 1B.Normal functionApical stereociliaVIIaDeafnessDisease phenotypeCochleaInherited diseaseRetinaShaker-1Permeation Properties and Differential Expression across the Auditory Receptor Epithelium of an Inward Rectifier K+ Channel Cloned from the Chick Inner Ear *
Navaratnam D, Escobar L, Covarrubias M, Oberholtzer J. Permeation Properties and Differential Expression across the Auditory Receptor Epithelium of an Inward Rectifier K+ Channel Cloned from the Chick Inner Ear *. Journal Of Biological Chemistry 1995, 270: 19238-19245. PMID: 7642595, DOI: 10.1074/jbc.270.33.19238.Peer-Reviewed Original ResearchMeSH KeywordsAmino Acid SequenceAnimalsBariumBase SequenceBasilar MembraneChickensCloning, MolecularDNA, ComplementaryEpitheliumEvoked PotentialsGene Expression RegulationGlutamic AcidHair Cells, AuditoryMolecular Sequence DataPotassium Channel BlockersPotassium ChannelsPotassium Channels, Inwardly RectifyingSequence Homology, Amino AcidXenopusConceptsAuditory receptor epitheliumDifferential expressionSingle-channel conductanceExcellent model systemSite-directed mutagenesisPutative pore regionIon channel genesInward rectifier potassium channelChick inner earCochlear sensory epitheliumReceptor epitheliumChick cochlear hair cellsChannel genesPrimary structureCochlear hair cellsNative channelsRectifier potassium channelSensory epitheliumExternal vestibulePore regionModel systemHair cellsPotassium channelsExpressionInward rectifier potassium current
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