A deafness mechanism of digenic Cx26 (GJB2) and Cx30 (GJB6) mutations: Reduction of endocochlear potential by impairment of heterogeneous gap junctional function in the cochlear lateral wall
Mei L, Chen J, Zong L, Zhu Y, Liang C, Jones R, Zhao H. A deafness mechanism of digenic Cx26 (GJB2) and Cx30 (GJB6) mutations: Reduction of endocochlear potential by impairment of heterogeneous gap junctional function in the cochlear lateral wall. Neurobiology Of Disease 2017, 108: 195-203. PMID: 28823936, PMCID: PMC5675824, DOI: 10.1016/j.nbd.2017.08.002.Peer-Reviewed Original ResearchConceptsCochlear lateral wallEndocochlear potentialHearing lossGap junctional functionDeafness mechanismLateral wallHeterozygous miceCx30 mutationsHair cell degenerationHomozygous knockout miceJunctional functionHeterozygous mouse modelGap junctionsOrgan of CortiSame gap junctional plaquesEP reductionFrequent causePathological changesMouse modelKnockout miceReceptor currentsCell degenerationNormal hearingHeterozygous mutationsMiceKv1.1 channelopathy abolishes presynaptic spike width modulation by subthreshold somatic depolarization
Vivekananda U, Novak P, Bello OD, Korchev YE, Krishnakumar SS, Volynski KE, Kullmann DM. Kv1.1 channelopathy abolishes presynaptic spike width modulation by subthreshold somatic depolarization. Proceedings Of The National Academy Of Sciences Of The United States Of America 2017, 114: 2395-2400. PMID: 28193892, PMCID: PMC5338558, DOI: 10.1073/pnas.1608763114.Peer-Reviewed Original ResearchConceptsSomatic depolarizationPotassium channelsAction potentialsPresynaptic potassium channelsPrimary hippocampal culturesSubthreshold membrane potential fluctuationsHeterozygous mouse modelEpisodic ataxia type 1Distinct potassium channelsSubthreshold modulationAxon transectionSmall boutonsCalcium influxHippocampal culturesMouse modelSynaptic boutonsKv1.1 subunitsMembrane potential fluctuationsNeurotransmitter releaseIntact axonsType 1Genetic deletionAtaxia type 1Further prolongationPresynaptic spikes
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