Transgenic expression of a dominant-negative connexin26 causes degeneration of the organ of Corti and non-syndromic deafness
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Life cycle of connexins in health and diseaseThe role of connexins in ear and skin physiology - functional insights from disease-associated mutationsFunctional analysis and regulation of purified connexin hemichannelsGenetics of hearing and deafnessAberrant 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.Mice with conditional deletion of Cx26 exhibit no vestibular phenotype despite secondary loss of Cx30 in the vestibular end organsGap junctions and cochlear homeostasisAssembly of the cochlear gap junction macromolecular complex requires connexin 26.The 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) DeficiencyACEMg Diet Supplement Modifies Progression of Hereditary Deafness.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.Calcium signaling in the cochlea - Molecular mechanisms and physiopathological implications.Viral vector tropism for supporting cells in the developing murine cochlea.The sound of silence: mouse models for hearing lossDefining the cellular environment in the organ of Corti following extensive hair cell loss: a basis for future sensory cell replacement in the Cochlea.Reduced electromotility of outer hair cells associated with connexin-related forms of deafness: an in silico study of a cochlear network mechanism.Connexin30 deficiency causes instrastrial fluid-blood barrier disruption within the cochlear stria vascularis.Inner ear drug delivery for auditory applications.Gap-junction channels dysfunction in deafness and hearing loss.ATP-mediated cell-cell signaling in the organ of Corti: the role of connexin channels.Orphan glutamate receptor delta1 subunit required for high-frequency hearing.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.Post-translational modifications of connexin26 revealed by mass spectrometry.Epithelial cell stretching and luminal acidification lead to a retarded development of stria vascularis and deafness in mice lacking pendrin.Connexin26 is responsible for anionic molecule permeability in the cochlea for intercellular signalling and metabolic communicationsTDP-43 potentiates alpha-synuclein toxicity to dopaminergic neurons in transgenic miceTemporal bone abnormalities in children with GJB2 mutationsDominant negative connexin26 mutation R75W causing severe hearing loss influences normal programmed cell death in postnatal organ of Corti.Deformation of the Outer Hair Cells and the Accumulation of Caveolin-2 in Connexin 26 Deficient MiceMolecular and physiological bases of the K+ circulation in the mammalian inner ear.Distinct Expression Patterns Of Causative Genes Responsible For Hereditary Progressive Hearing Loss In Non-Human Primate Cochlea.Ca2+ signaling in the inner ear.Cochleosaccular dysplasia associated with a connexin 26 mutation in keratitis-ichthyosis-deafness syndrome.Diverse deafness mechanisms of connexin mutations revealed by studies using in vitro approaches and mouse models.Connexin-26 mutations in deafness and skin disease.Ultrastructural pathological changes in the cochlear cells of connexin 26 conditional knockout mice.
P2860
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P2860
Transgenic expression of a dominant-negative connexin26 causes degeneration of the organ of Corti and non-syndromic deafness
description
2003 nî lūn-bûn
@nan
2003 թուականի Մայիսին հրատարակուած գիտական յօդուած
@hyw
2003 թվականի մայիսին հրատարակված գիտական հոդված
@hy
2003年の論文
@ja
2003年論文
@yue
2003年論文
@zh-hant
2003年論文
@zh-hk
2003年論文
@zh-mo
2003年論文
@zh-tw
2003年论文
@wuu
name
Transgenic expression of a dom ...... rti and non-syndromic deafness
@ast
Transgenic expression of a dom ...... rti and non-syndromic deafness
@en
Transgenic expression of a dom ...... rti and non-syndromic deafness
@nl
type
label
Transgenic expression of a dom ...... rti and non-syndromic deafness
@ast
Transgenic expression of a dom ...... rti and non-syndromic deafness
@en
Transgenic expression of a dom ...... rti and non-syndromic deafness
@nl
prefLabel
Transgenic expression of a dom ...... rti and non-syndromic deafness
@ast
Transgenic expression of a dom ...... rti and non-syndromic deafness
@en
Transgenic expression of a dom ...... rti and non-syndromic deafness
@nl
P2093
P356
P1476
Transgenic expression of a dom ...... rti and non-syndromic deafness
@en
P2093
Akiko Ichinohe
An-Ping Xia
Kanako Kojima
Katsuhisa Ikeda
Masaaki Suzuki
Shigeo Kure
Takayuki Kudo
Toshimitsu Kobayashi
Yoichi Matsubara
Yoichi Suzuki
P304
P356
10.1093/HMG/DDG116
P407
P577
2003-05-01T00:00:00Z