Search results for "Hair Cells"

showing 10 items of 30 documents

Genome-wide association analysis on normal hearing function identifies PCDH20 and SLC28A3 as candidates for hearing function and loss.

2015

Hearing loss and individual differences in normal hearing both have a substantial genetic basis. Although many new genes contributing to deafness have been identified, very little is known about genes/variants modulating the normal range of hearing ability. To fill this gap, we performed a two-stage meta-analysis on hearing thresholds (tested at 0.25, 0.5, 1, 2, 4, 8 kHz) and on pure-tone averages (low-, medium-and high-frequency thresholds grouped) in several isolated populations from Italy and Central Asia (total N = 2636). Here, we detected two genome-wide significant loci close to PCDH20 and SLC28A3 (top hits: rs78043697, P = 4.71E-10 and rs7032430, P = 2.39E-09, respectively). For both…

Genome-wide association studieLOCICOMMON DISEASESNerve Tissue ProteinsVARIANTSSUSCEPTIBILITYDeafnessGenome-wide association studiesMiceHearingGenome-wide association studies; normal hearing function; PCDH20; SLC28A3PCDH20SLC28A3otorhinolaryngologic diseasesAnimalsHumansGenetic Predisposition to DiseaseMETAANALYSISHair Cells Auditory InnerSequence Analysis RNAAssociation Studies ArticlesMembrane Transport ProteinsLOCALIZATIONCadherinsTRANSPORTER-3ProtocadherinsGENOTYPEMYOSIN-VIIAItalyAsia Centralnormal hearing function3111 BiomedicineGenome-Wide Association StudyHuman molecular genetics
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Localization of Ca(2+)-stores and tissue compartments with a Ca(2+)-binding capacity in the organ of Corti of the guinea-pig by electron energy-loss …

1992

SUMMARY The addition of 10 mM CaCl2 to glutaraldehyde fixative leads to the formation of small electron-dense deposits in the organ of Corti of the guinea-pig. These precipitates are mainly attached to cell membranes in contact with different extracellular lymphatic fluids. A higher number of precipitates is localized in the acellular parts of tectorial and basilar membrane. Electron energy-loss spectroscopy (EELS) was used to determine the elemental composition of the deposits formed. The spectra showed a prominent signal at the Ca2+ L2,3 ionization edge. Oxygen could also be detected in all the precipitates analysed. EELS analysis of mitochondria of the inner and outer hair cells after co…

HistologyGuinea PigsAnalytical chemistrychemistry.chemical_elementCalciumPathology and Forensic Medicinechemistry.chemical_compoundCalcium ChlorideHair Cells AuditorymedicineExtracellularAnimalsInner earOrgan of CortiFixativeOrganellesElectron energy loss spectroscopyMitochondriaOxygenMicroscopy ElectronMembranemedicine.anatomical_structurechemistryOrgan of CortiBiophysicsCalciumGlutaraldehydeElectron Probe MicroanalysisJournal of microscopy
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Possible Ca 2+ -dependent mechanism of apical outer hair cell modulation within the cochlea of the guinea pig

1998

Calcium ions were precipitated with potassium antimonate after injection of the inorganic calcium channel blocker MnCl2 or the inorganic potassium channel blockers BaCl2 or CsCl into the perilymph of the scala vestibuli of the guinea pig. The spatial distribution of the formed histochemical reaction products within the organ of Corti was studied by energy-filtering transmission-electron microscopy. Compared with untreated control ears, the number of the formed precipitates drastically increased at the extracellular side of the lamina reticularis after application of the various inorganic channel blockers. The apical side of the outer hair cells and the intervening Deiter cells were covered …

HistologyTectorial membraneBarium CompoundsGuinea PigsCesiumchemistry.chemical_elementPerilymphCalciumPathology and Forensic MedicineChloridesmedicineAnimalsChemical PrecipitationChannel blockerCochleaLamina reticularisCell BiologyAnatomyCalcium Channel BlockersPerilymphHair Cells Auditory OuterMicroscopy Electronmedicine.anatomical_structurechemistryOrgan of CortiBiophysicsCalciumHair cellElectron Probe MicroanalysisCell and Tissue Research
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Vezatin, a novel transmembrane protein, bridges myosin VIIA to the cadherin-catenins complex

2000

International audience; Defects in myosin VIIA are responsible for deafness in the human and mouse. The role of this unconventional myosin in the sensory hair cells of the inner ear is not yet understood. Here we show that the C-terminal FERM domain of myosin VIIA binds to a novel transmembrane protein, vezatin, which we identi®ed by a yeast two-hybrid screen. Vezatin is a ubiquitous protein of adherens cell±cell junctions, where it interacts with both myosin VIIA and the cadherin±catenins complex. Its recruitment to adherens junctions implicates the C-terminal region of a-catenin. Taken together, these data suggest that myosin VIIA, anchored by vezatin to the cadherin±catenins complex, cre…

MESH: Cytoskeletal ProteinsMESH: alpha CateninStereocilia (inner ear)[SDV]Life Sciences [q-bio]MESH: Amino Acid SequenceDeafnessMESH: CadherinsMiceMESH: Protein Structure Tertiary0302 clinical medicine[SDV.MHEP.MI]Life Sciences [q-bio]/Human health and pathology/Infectious diseasesMyosinMESH: Hair Cells AuditoryMESH: AnimalsCytoskeleton0303 health sciencesFERM domainGeneral NeuroscienceMESH: Alternative SplicingArticlesCadherinsCell biologymedicine.anatomical_structureIntercellular Junctions[SDV.MP]Life Sciences [q-bio]/Microbiology and ParasitologyMyosin VIIaHair cellMESH: Membrane ProteinsMESH: DyneinsProtein BindingMESH: MutationMacromolecular SubstancesMolecular Sequence DataMESH: Deafnessmacromolecular substancesBiologyIn Vitro TechniquesMyosinsGeneral Biochemistry Genetics and Molecular BiologyCell LineAdherens junction03 medical and health sciencesHair Cells Auditorymedicineotorhinolaryngologic diseasesAnimalsHumansMESH: Myosin VIIaMESH: Protein BindingAmino Acid SequenceMolecular BiologyMESH: Mice030304 developmental biologyMESH: In Vitro TechniquesMESH: Molecular Sequence DataMESH: HumansGeneral Immunology and MicrobiologyCadherinDyneinsMembrane ProteinsMESH: Macromolecular SubstancesMESH: MyosinsActin cytoskeleton[SDV.MP.BAC]Life Sciences [q-bio]/Microbiology and Parasitology/BacteriologyProtein Structure TertiaryMESH: Cell LineAlternative SplicingCytoskeletal ProteinsMutationsense organs030217 neurology & neurosurgeryalpha Catenin[SDV.MHEP]Life Sciences [q-bio]/Human health and pathologyMESH: Intercellular Junctions
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Histochemical localization of calcium ATPase in the cochlea of the guinea pig

1992

The activity of Ca(2+)-ATPase in the inner ear of the guinea pig was studied ultracytochemically by the lead citrate reaction. The electron-dense reaction products as an expression of Ca(2+)-ATPase activity were localized in endolymphatic cells of Reissner's membrane, in outer and inner hair cells and in some supporting cells. The main finding was the difference in the localization of Ca(2+)-ATPase in outer and inner hair cells. In the latter cells the activity sites were mainly intracellular and in apical membrane specializations, whereas in the outer hair cells the enzyme was localized in the apical membrane specializations and the basolateral plasma membrane.

MaleATPaseGuinea PigsCalcium-Transporting ATPasesGuinea pigEndolymphHair Cells AuditorymedicineAnimalsInner earOrgan of CortiCochleabiologyHistocytochemistryGeneral MedicineBasolateral plasma membraneApical membraneCochleaCalcium ATPasemedicine.anatomical_structureOtorhinolaryngologyBiochemistryBiophysicsbiology.proteinFemaleCalcium Channelssense organsIntracellularEuropean Archives of Oto-Rhino-Laryngology
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USH3A transcripts encode clarin-1, a four-transmembrane-domain protein with a possible role in sensory synapses.

2002

Usher syndrome type 3 (USH3) is an autosomal recessive disorder characterised by the association of post-lingual progressive hearing loss, progressive visual loss due to retinitis pigmentosa and variable presence of vestibular dysfunction. Because the previously defined transcripts do not account for all USH3 cases, we performed further analysis and revealed the presence of additional exons embedded in longer human and mouse USH3A transcripts and three novel USH3A mutations. Expression of Ush3a transcripts was localised by whole mount in situ hybridisation to cochlear hair cells and spiral ganglion cells. The full length USH3A transcript encodes clarin-1, a four-transmembrane-domain protein…

MaleUsher syndromeMolecular Sequence DataBiologyPhotoreceptor cellSynapse03 medical and health sciencesExonMice0302 clinical medicineSequence Analysis ProteinRetinitis pigmentosaHair Cells Auditoryotorhinolaryngologic diseasesGeneticsmedicineAnimalsHumansAmino Acid SequenceGenetics (clinical)Spiral ganglionIn Situ HybridizationPhylogeny030304 developmental biology0303 health sciencesGene Expression ProfilingChromosome MappingMembrane ProteinsSequence Analysis DNAmedicine.diseaseCell biologyPedigreeTransmembrane domainmedicine.anatomical_structureMutationSynapsesFemalesense organsHair cellCalcium ChannelsSequence Alignment030217 neurology & neurosurgeryEuropean journal of human genetics : EJHG
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Auditory Neuropathy in Children

2001

Auditory neuropathy is a sensorineural disorder characterized by absent or abnormal auditory brainstem evoked potentials and normal cochlear outer hair cell function. A variety of processes is thought to be involved in its pathophysiology and their influence on hearing may be different. We present here the diagnostic sequence and management of two new cases of auditory neuropathy in breastfeeding children.

Malemedicine.medical_specialtyHearing lossHearing Loss SensorineuralOtoacoustic Emissions SpontaneousAuditory neuropathyAudiologyEvoked Potentials Auditory Brain StemVestibulocochlear Nerve Diseasesotorhinolaryngologic diseasesmedicineHumansCochlear Nervebusiness.industryAuditory Perceptual DisordersFollow up studiesInfantGeneral Medicinemedicine.diseasePathophysiologyHair Cells Auditory Outermedicine.anatomical_structureAcoustic StimulationOtorhinolaryngologyFemaleBrainstemHair cellmedicine.symptombusinessBrain StemFollow-Up StudiesActa Oto-Laryngologica
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An experimental comparative study of dexamethasone, melatonin and tacrolimus in noise-induced hearing loss.

2008

The calcineurin inhibitor tacrolimus (TCR) and the pineal gland hormone and antioxidant melatonin (MLT) have been shown to possess otoprotective properties against noise-induced hearing loss (NIHL). In contrast, dexamethasone (DXM) was not effective as an otoprotective agent against NIHL. Further studies are needed to understand the exact molecular mechanisms involved.Exposure to noise pollution and use of audio devices for long periods of time at high volume is known to cause hearing loss or NIHL. Our goal was to evaluate the effectiveness of various known compounds such as the anti-inflammatory DXM, the antioxidant MLT and the immunosuppressant TCR against NIHL.Thirty-two Wistar rats were…

Malemedicine.medical_specialtyHearing lossOtoacoustic Emissions SpontaneousAnti-Inflammatory AgentsAntioxidantsDexamethasoneTacrolimusMelatoninPineal glandInternal medicineotorhinolaryngologic diseasesmedicineEvoked Potentials Auditory Brain StemAnimalsRats WistarDexamethasoneMelatoninbusiness.industryReverse Transcriptase Polymerase Chain ReactionTumor Necrosis Factor-alphaGeneral Medicinemedicine.diseaseTacrolimusRatsCalcineurinHair Cells Auditory OuterEndocrinologymedicine.anatomical_structureOtorhinolaryngologyHearing Loss Noise-Inducedmedicine.symptombusinessProto-Oncogene Proteins c-fosNoise-induced hearing lossImmunosuppressive AgentsHormonemedicine.drugActa oto-laryngologica
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A theoretical basis for the high-frequency performance of the outer hair cell’s receptor potential

1997

The frequency response of the outer hair cell (OHC) was studied theoretically. An electrical model of the OHC was analyzed mathematically, taking into account the effect of its inherent voltage-dependent capacitance. It was found that the variations of the capacitance dependent on the membrane potential could enhance the high-frequency response of the OHC, so that its cutoff frequency could be extended into the audio range. It was found further that the enhancement of the frequency response of the OHC was strongly dependent on its resting potential and on the ratio of the maximum voltage-dependent capacitance to the membrane linear capacitance.

Models AnatomicMembrane potentialFrequency responseMaterials scienceAcoustics and UltrasonicsAcousticsReceptor potentialResting potentialCapacitanceCutoff frequencymedicine.anatomical_structureMembraneArts and Humanities (miscellaneous)Hair Cells AuditoryBiophysicsmedicineHumanssense organsHair cellThe Journal of the Acoustical Society of America
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Immunoelectron-microscopic localization of synaptophysin in the organ of Corti of the guinea pig.

1997

Synaptophysin has been localized previously in the mammalian cochlea at the light-microscopic level and in few reports by electron microscopy using either a preincubation procedure or the avidin-biotin reaction. Here we present results of the electron-microscopic analysis for postembedding immunoreactivity of synaptophysin in the cochlea of the guinea pig of LR-White-embedded samples. Strong synaptophysin immunoreactivity is located in the cytoplasm of the efferent nerve endings at the base of inner and outer hair cells. Besides this, some antibodies to synaptophysin were also identified in the cytoplasm of outer hair cells. To get more information about the cellular content of synaptophysi…

Pathologymedicine.medical_specialtyCytoplasmImmunoelectron microscopyImmunocytochemistryGuinea PigsSynaptophysinlaw.inventionGuinea piglawotorhinolaryngologic diseasesmedicineAnimalsInner earMicroscopy ImmunoelectronCochleaHair Cells Auditory InnerbiologyChemistryImmunohistochemistryHair Cells Auditory Outermedicine.anatomical_structurenervous systemOtorhinolaryngologyOrgan of CortiSynaptophysinbiology.proteinsense organsElectron microscopeORL; journal for oto-rhino-laryngology and its related specialties
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