Perinatal Gjb2 gene transfer rescues hearing in a mouse model of hereditary deafness (Q42153064)

17 October 2017

title

Perinatal Gjb2 gene transfer rescues hearing in a mouse model of hereditary deafness (English)

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17 October 2017

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Takashi Iizuka

1

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17 October 2017

Kazusaku Kamiya

2

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17 October 2017

Satoru Gotoh

3

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17 October 2017

Yoshinobu Sugitani

4

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17 October 2017

Masaaki Suzuki

5

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17 October 2017

Tetsuo Noda

6

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17 October 2017

Osamu Minowa

7

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17 October 2017

Katsuhisa Ikeda

8

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17 October 2017

publication date

23 March 2015

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17 October 2017

published in

Human Molecular Genetics

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17 October 2017

volume

24

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17 October 2017

issue

13

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17 October 2017

page(s)

3651-3661

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Molecular genetics of hearing loss

17 October 2017

cites work

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Profound deafness in childhood

21 January 2018

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In vitro and in vivo suppression of GJB2 expression by RNA interference.

21 January 2018

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Restoration of hearing in the VGLUT3 knockout mouse using virally mediated gene therapy.

21 January 2018

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Rescue of hearing and vestibular function by antisense oligonucleotides in a mouse model of human deafness

21 January 2018

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Mouse otocyst transuterine gene transfer restores hearing in mice with connexin 30 deletion-associated hearing loss.

21 January 2018

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Virally expressed connexin26 restores gap junction function in the cochlea of conditional Gjb2 knockout mice

21 January 2018

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Targeted ablation of connexin26 in the inner ear epithelial gap junction network causes hearing impairment and cell death

21 January 2018

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Transgenic expression of a dominant-negative connexin26 causes degeneration of the organ of Corti and non-syndromic deafness

21 January 2018

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Postnatal development of the organ of Corti in dominant-negative Gjb2 transgenic mice

21 January 2018

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Cochlear outer hair cells in a dominant-negative connexin26 mutant mouse preserve non-linear capacitance in spite of impaired distortion product otoacoustic emission

21 January 2018

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Targeted connexin26 ablation arrests postnatal development of the organ of Corti

21 January 2018

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Assembly of the cochlear gap junction macromolecular complex requires connexin 26.

21 January 2018

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Treatment of peripheral sensorineural hearing loss: gene therapy

21 January 2018

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Noninvasive in vivo delivery of transgene via adeno-associated virus into supporting cells of the neonatal mouse cochlea

21 January 2018

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Transplacental Uptake of Glucose Is Decreased in Embryonic Lethal Connexin26-deficient Mice

21 January 2018

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Apoptosis in neural crest cells by functional loss of APC tumor suppressor gene

21 January 2018

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The endocochlear potential depends on two K diffusion potentials and an electrical barrier in the stria vascularis of the inner ear

21 January 2018

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Deafness in Claudin 11-null mice reveals the critical contribution of basal cell tight junctions to stria vascularis function.

21 January 2018

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Compartmentalization established by claudin-11-based tight junctions in stria vascularis is required for hearing through generation of endocochlear potential.

21 January 2018

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Supporting sensory transduction: cochlear fluid homeostasis and the endocochlear potential

21 January 2018

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Direct measurement of longitudinal endolymph flow rate in the guinea pig cochlea

21 January 2018

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In vivo delivery of recombinant viruses to the fetal murine cochlea: transduction characteristics and long-term effects on auditory function

21 January 2018

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Gene transfer in inner ear cells: a challenging race

21 January 2018

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Gap junctions in the rat cochlea: immunohistochemical and ultrastructural analysis

21 January 2018

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K cycling and the endocochlear potential

21 January 2018

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Impaired permeability to Ins(1,4,5)P3 in a mutant connexin underlies recessive hereditary deafness

21 January 2018

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Gap junction-mediated intercellular biochemical coupling in cochlear supporting cells is required for normal cochlear functions

21 January 2018

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Gap junction mediated intercellular metabolite transfer in the cochlea is compromised in connexin30 null mice

21 January 2018

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ATP-mediated cell-cell signaling in the organ of Corti: the role of connexin channels

21 January 2018

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Gap junctions in the inner ear: comparison of distribution patterns in different vertebrates and assessement of connexin composition in mammals

21 January 2018

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Human connexin26 and connexin30 form functional heteromeric and heterotypic channels

21 January 2018

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Connexin30 null and conditional connexin26 null mice display distinct pattern and time course of cellular degeneration in the cochlea

21 January 2018

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Restoration of connexin26 protein level in the cochlea completely rescues hearing in a mouse model of human connexin30-linked deafness

21 January 2018

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Hearing is normal without connexin30

21 January 2018

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BAAV mediated GJB2 gene transfer restores gap junction coupling in cochlear organotypic cultures from deaf Cx26Sox10Cre mice

21 January 2018

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Down regulated connexin26 at different postnatal stage displayed different types of cellular degeneration and formation of organ of Corti.

21 January 2018

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Timed conditional null of connexin26 in mice reveals temporary requirements of connexin26 in key cochlear developmental events before the onset of hearing

21 January 2018

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Intracellular domains of mouse connexin26 and -30 affect diffusional and electrical properties of gap junction channels

21 January 2018

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Connexin32 can restore hearing in connexin26 deficient mice

21 January 2018

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Rapid Colorectal Adenoma Formation Initiated by Conditional Targeting of the Apc Gene

21 January 2018

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Altered cochlear fibrocytes in a mouse model of DFN3 nonsyndromic deafness.

21 January 2018

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21 January 2018

Identifiers

DOI

10.1093/HMG/DDV109

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17 October 2017

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