Targeted connexin26 ablation arrests postnatal development of the organ of Corti.
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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.Aberrant Cx26 hemichannels and keratitis-ichthyosis-deafness syndrome: insights into syndromic hearing lossCellular and Deafness Mechanisms Underlying Connexin Mutation-Induced Hearing Loss - A Common Hereditary Deafness.Hypothesis of K+-Recycling Defect Is Not a Primary Deafness Mechanism for Cx26 (GJB2) DeficiencyPannexin 1 deficiency can induce hearing lossReduced 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.Active cochlear amplification is dependent on supporting cell gap junctions7,8,3'-Trihydroxyflavone, a potent small molecule TrkB receptor agonist, protects spiral ganglion neurons from degeneration both in vitro and in vivoDefining the cellular environment in the organ of Corti following extensive hair cell loss: a basis for future sensory cell replacement in the Cochlea.Cell degeneration is not a primary causer for Connexin26 (GJB2) deficiency associated hearing loss.Characterization of a knock-in mouse model of the homozygous p.V37I variant in Gjb2.Epithelial cell stretching and luminal acidification lead to a retarded development of stria vascularis and deafness in mice lacking pendrin.Amplification mode differs along the length of the mouse cochlea as revealed by connexin 26 deletion from specific gap junctionsApoptosis Progression in the Hair Cells in the Organ of Corti of GJB2 Conditional Knockout Mice.Hearing loss associated with enlargement of the vestibular aqueduct: mechanistic insights from clinical phenotypes, genotypes, and mouse models.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 MiceConnexin-26 mutations in deafness and skin disease.Ultrastructural pathological changes in the cochlear cells of connexin 26 conditional knockout mice.GJB2-associated hearing loss: systematic review of worldwide prevalence, genotype, and auditory phenotype.Connexins and gap junctions in the inner ear--it's not just about K⁺ recyclingMechanisms linking connexin mutations to human diseases.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.Gap junction mediated miRNA intercellular transfer and gene regulation: A novel mechanism for intercellular genetic communication.Connexin26 gap junction mediates miRNA intercellular genetic communication in the cochlea and is required for inner ear development.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.Perinatal Gjb2 gene transfer rescues hearing in a mouse model of hereditary deafness.Altered CO2 sensitivity of connexin26 mutant hemichannels in vitroDominant Cx26 mutants associated with hearing loss have dominant-negative effects on wild type Cx26.Early 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.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.Connexin hemichannels and cochlear function.
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P2860
Targeted connexin26 ablation arrests postnatal development of the organ of Corti.
description
article científic
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article scientifique
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articolo scientifico
@it
artigo científico
@pt
bilimsel makale
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scientific article published on 09 May 2009
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vedecký článok
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vetenskaplig artikel
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videnskabelig artikel
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vědecký článek
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name
Targeted connexin26 ablation arrests postnatal development of the organ of Corti.
@en
Targeted connexin26 ablation arrests postnatal development of the organ of Corti.
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type
label
Targeted connexin26 ablation arrests postnatal development of the organ of Corti.
@en
Targeted connexin26 ablation arrests postnatal development of the organ of Corti.
@nl
prefLabel
Targeted connexin26 ablation arrests postnatal development of the organ of Corti.
@en
Targeted connexin26 ablation arrests postnatal development of the organ of Corti.
@nl
P2093
P2860
P1476
Targeted connexin26 ablation arrests postnatal development of the organ of Corti.
@en
P2093
Binfei Zhou
Qing Chang
Wenxue Tang
Yunfeng Wang
P2860
P356
10.1016/J.BBRC.2009.05.023
P407
P577
2009-05-09T00:00:00Z