Connexin30 null and conditional connexin26 null mice display distinct pattern and time course of cellular degeneration in the cochlea
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The role of connexins in ear and skin physiology - functional insights from disease-associated mutationsGenetics of hearing and deafnessConditional gene expression in the mouse inner ear using Cre-loxP.Cellular and Deafness Mechanisms Underlying Connexin Mutation-Induced Hearing Loss - A Common Hereditary Deafness.Mice with conditional deletion of Cx26 exhibit no vestibular phenotype despite secondary loss of Cx30 in the vestibular end organsThe human deafness-associated connexin 30 T5M mutation causes mild hearing loss and reduces biochemical coupling among cochlear non-sensory cells in knock-in mice.Hypothesis of K+-Recycling Defect Is Not a Primary Deafness Mechanism for Cx26 (GJB2) DeficiencyReduced Connexin26 in the Mature Cochlea Increases Susceptibility to Noise-Induced Hearing Lossin MiceConnexin26 (GJB2) deficiency reduces active cochlear amplification leading to late-onset hearing loss.Connexin 26 null mice exhibit spiral ganglion degeneration that can be blocked by BDNF gene therapy.Virally expressed connexin26 restores gap junction function in the cochlea of conditional Gjb2 knockout mice.Protection of spiral ganglion neurons from degeneration using small-molecule TrkB receptor agonists.Selective ablation of pillar and deiters' cells severely affects cochlear postnatal development and hearing in mice.7,8,3'-Trihydroxyflavone, a potent small molecule TrkB receptor agonist, protects spiral ganglion neurons from degeneration both in vitro and in vivoCurrent status and prospects of gene therapy for the inner earCalcium signaling in the cochlea - Molecular mechanisms and physiopathological implications.BAAV mediated GJB2 gene transfer restores gap junction coupling in cochlear organotypic cultures from deaf Cx26Sox10Cre mice.ATP-mediated cell-cell signaling in the organ of Corti: the role of connexin channels.Cell degeneration is not a primary causer for Connexin26 (GJB2) deficiency associated hearing loss.Mouse otocyst transuterine gene transfer restores hearing in mice with connexin 30 deletion-associated hearing loss.The role of gap junction channels during physiologic and pathologic conditions of the human central nervous systemIncreased p66Shc in the inner ear of D-galactose-induced aging mice with accumulation of mitochondrial DNA 3873-bp deletion: p66Shc and mtDNA damage in the inner ear during aging.Early developmental expression of connexin26 in the cochlea contributes to its dominate functional role in the cochlear gap junctions.Deformation of the Outer Hair Cells and the Accumulation of Caveolin-2 in Connexin 26 Deficient MiceVirally mediated Kcnq1 gene replacement therapy in the immature scala media restores hearing in a mouse model of human Jervell and Lange-Nielsen deafness syndrome.Targeted connexin26 ablation arrests postnatal development of the organ of Corti.Key functions for gap junctions in skin and hearing.Mechanisms linking connexin mutations to human diseases.Dominant connexin26 mutants associated with human hearing loss have trans-dominant effects on connexin30.Deafness induced by Connexin 26 (GJB2) deficiency is not determined by endocochlear potential (EP) reduction but is associated with cochlear developmental disorders.Kölliker's organ and the development of spontaneous activity in the auditory system: implications for hearing dysfunction.Perinatal Gjb2 gene transfer rescues hearing in a mouse model of hereditary deafness.Altered CO2 sensitivity of connexin26 mutant hemichannels in vitroEarly postnatal virus inoculation into the scala media achieved extensive expression of exogenous green fluorescent protein in the inner ear and preserved auditory brainstem response thresholds.Mouse Panx1 Is Dispensable for Hearing Acquisition and Auditory Function.Chronic prenatal hypoxia impairs cochlear development, a mechanism involving connexin26 expression and promoter methylation.Developmental abnormalities in supporting cell phalangeal processes and cytoskeleton in the GJB2 knockdown mouse model.Novel connexin 30 and connexin 26 mutational spectrum in patients with progressive sensorineural hearing loss.Cochlear Gene Therapy for Sensorineural Hearing Loss: Current Status and Major Remaining Hurdles for Translational Success.Ablation of connexin30 in transgenic mice alters expression patterns of connexin26 and connexin32 in glial cells and leptomeninges
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Connexin30 null and conditional connexin26 null mice display distinct pattern and time course of cellular degeneration in the cochlea
description
2009 nî lūn-bûn
@nan
2009 թուականի Հոկտեմբերին հրատարակուած գիտական յօդուած
@hyw
2009 թվականի հոտեմբերին հրատարակված գիտական հոդված
@hy
2009年の論文
@ja
2009年論文
@yue
2009年論文
@zh-hant
2009年論文
@zh-hk
2009年論文
@zh-mo
2009年論文
@zh-tw
2009年论文
@wuu
name
Connexin30 null and conditiona ...... ar degeneration in the cochlea
@ast
Connexin30 null and conditiona ...... ar degeneration in the cochlea
@en
Connexin30 null and conditiona ...... ar degeneration in the cochlea
@nl
type
label
Connexin30 null and conditiona ...... ar degeneration in the cochlea
@ast
Connexin30 null and conditiona ...... ar degeneration in the cochlea
@en
Connexin30 null and conditiona ...... ar degeneration in the cochlea
@nl
prefLabel
Connexin30 null and conditiona ...... ar degeneration in the cochlea
@ast
Connexin30 null and conditiona ...... ar degeneration in the cochlea
@en
Connexin30 null and conditiona ...... ar degeneration in the cochlea
@nl
P2093
P2860
P356
P1476
Connexin30 null and conditiona ...... ar degeneration in the cochlea
@en
P2093
Qing Chang
Wenxue Tang
Yunfeng Wang
P2860
P304
P356
10.1002/CNE.22117
P407
P577
2009-10-01T00:00:00Z