Cochlear Gene Therapy for Sensorineural Hearing Loss: Current Status and Major Remaining Hurdles for Translational Success. (Q55657184)

http://europepmc.org/abstract/PMC/6028713

19 July 2018

title

Cochlear Gene Therapy for Sensorineural Hearing Loss: Current Status and Major Remaining Hurdles for Translational Success. (English)

1 reference

http://europepmc.org/abstract/PMC/6028713

19 July 2018

0 references

1 reference

sensorineural hearing loss

1 reference

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:29997477%20AND%20SRC:MED&resulttype=core&format=json

7 June 2020

Wenjuan Zhang

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:29997477%20AND%20SRC:MED&resulttype=core&format=json

7 June 2020

Sun Myoung Kim

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:29997477%20AND%20SRC:MED&resulttype=core&format=json

7 June 2020

Wenwen Wang

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:29997477%20AND%20SRC:MED&resulttype=core&format=json

7 June 2020

Cuiyuan Cai

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:29997477%20AND%20SRC:MED&resulttype=core&format=json

7 June 2020

Yong Feng

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:29997477%20AND%20SRC:MED&resulttype=core&format=json

7 June 2020

Xi Lin

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:29997477%20AND%20SRC:MED&resulttype=core&format=json

7 June 2020

publication date

26 June 2018

1 reference

http://europepmc.org/abstract/PMC/6028713

19 July 2018

published in

Frontiers in Molecular Neuroscience

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:29997477%20AND%20SRC:MED&resulttype=core&format=json

7 June 2020

volume

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:29997477%20AND%20SRC:MED&resulttype=core&format=json

7 June 2020

page(s)

221

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:29997477%20AND%20SRC:MED&resulttype=core&format=json

Creative Commons Attribution 4.0 International

7 June 2020

start time

26 June 2018

1 reference

April 2022 Public Data File from Crossref

copyrighted

0 references

cites work

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Comprehensive molecular etiology analysis of nonsyndromic hearing impairment from typical areas in China

27 July 2018

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

27 July 2018

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27 July 2018

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27 July 2018

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27 July 2018

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27 July 2018

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27 July 2018

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Sensorineural deafness and seizures in mice lacking vesicular glutamate transporter 3.

27 July 2018

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Functional significance of channels and transporters expressed in the inner ear and kidney

27 July 2018

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Ectopic expression of a microbial-type rhodopsin restores visual responses in mice with photoreceptor degeneration

27 July 2018

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Cochlear gap junctions coassembled from Cx26 and 30 show faster intercellular Ca2+ signaling than homomeric counterparts.

27 July 2018

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27 July 2018

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

27 July 2018

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Connexin30 (Gjb6)-deficiency causes severe hearing impairment and lack of endocochlear potential

27 July 2018

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A mutation in OTOF, encoding otoferlin, a FER-1-like protein, causes DFNB9, a nonsyndromic form of deafness

27 July 2018

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The deafness-associated mitochondrial DNA mutation at position 7445, which affects tRNASer(UCN) precursor processing, has long-range effects on NADH dehydrogenase subunit ND6 gene expression.

27 July 2018

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12 August 2018

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12 August 2018

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12 August 2018

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Genetics of Nonsyndromic Congenital Hearing Loss

12 August 2018

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12 August 2018

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12 August 2018

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

12 August 2018

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12 August 2018

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Close-field electroporation gene delivery using the cochlear implant electrode array enhances the bionic ear.

12 August 2018

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Deafness induced by Connexin 26 (GJB2) deficiency is not determined by endocochlear potential (EP) reduction but is associated with cochlear developmental disorders

12 August 2018

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12 August 2018

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Optogenetic stimulation of the auditory pathway.

12 August 2018

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TMC function in hair cell transduction

12 August 2018

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Three deaf mice: mouse models for TECTA-based human hereditary deafness reveal domain-specific structural phenotypes in the tectorial membrane

12 August 2018

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

12 August 2018

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Mid-stage intervention achieves similar efficacy as conventional early-stage treatment using gene therapy in a pre-clinical model of retinitis pigmentosa

12 August 2018

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Hair cell overexpression of Islet1 reduces age-related and noise-induced hearing loss

12 August 2018

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Therapeutic margins in a novel preclinical model of retinitis pigmentosa.

12 August 2018

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SLC26A4 targeted to the endolymphatic sac rescues hearing and balance in Slc26a4 mutant mice.

12 August 2018

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TMC1 and TMC2 are components of the mechanotransduction channel in hair cells of the mammalian inner ear

12 August 2018

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Advancing genetic testing for deafness with genomic technology

12 August 2018

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Retinal optogenetic therapies: clinical criteria for candidacy

12 August 2018

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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

12 August 2018

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

12 August 2018

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Gene transfer in inner ear cells: a challenging race

12 August 2018

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Applications of targeted gene capture and next-generation sequencing technologies in studies of human deafness and other genetic disabilities.

12 August 2018

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Mechanotransduction in mouse inner ear hair cells requires transmembrane channel-like genes

12 August 2018

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Deafness in the genomics era

12 August 2018

1 reference

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Mouse model of enlarged vestibular aqueducts defines temporal requirement of Slc26a4 expression for hearing acquisition

12 August 2018

1 reference

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Targeted genomic capture and massively parallel sequencing to identify genes for hereditary hearing loss in Middle Eastern families

12 August 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=6028713

Gene therapy for leber congenital amaurosis caused by RPE65 mutations: safety and efficacy in 15 children and adults followed up to 3 years

12 August 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=6028713

Hereditary hearing loss: from human mutation to mechanism

12 August 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=6028713

Nerve maintenance and regeneration in the damaged cochlea

12 August 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=6028713

Overexpression of X-linked inhibitor of apoptosis protein protects against noise-induced hearing loss in mice

12 August 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=6028713

Stereocilin connects outer hair cell stereocilia to one another and to the tectorial membrane

12 August 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=6028713

Comprehensive genetic testing for hereditary hearing loss using massively parallel sequencing.

12 August 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=6028713

Efficient transgene reconstitution with hybrid dual AAV vectors carrying the minimized bridging sequences

12 August 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=6028713

Transgenic BDNF induces nerve fiber regrowth into the auditory epithelium in deaf cochleae

12 August 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=6028713

12 August 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=6028713

Quo vadis, hair cell regeneration?

12 August 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=6028713

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

12 August 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=6028713

Loss of cochlear HCO3- secretion causes deafness via endolymphatic acidification and inhibition of Ca2+ reabsorption in a Pendred syndrome mouse model

12 August 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=6028713

Comprehensive classification of the auditory sensory projections in the brain of the fruit fly Drosophila melanogaster

12 August 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=6028713

Mutations in the gene encoding pejvakin, a newly identified protein of the afferent auditory pathway, cause DFNB59 auditory neuropathy

12 August 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=6028713

Sensorineural hearing loss in children.

12 August 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=6028713

Congenital deaf-mutism, functional heart disease with prolongation of the Q-T interval and sudden death

12 August 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=6028713

Current issues in cochlear gene transfer

12 August 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=6028713

Deafness and renal tubular acidosis in mice lacking the K-Cl co-transporter Kcc4.

12 August 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=6028713

Gene therapy restores vision in a canine model of childhood blindness

12 August 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=6028713

Targeted disruption of the Kcnq1 gene produces a mouse model of Jervell and Lange-Nielsen Syndrome.

12 August 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=6028713

A genetic approach to understanding auditory function

12 August 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=6028713

Mutations in Cdh23, encoding a new type of cadherin, cause stereocilia disorganization in waltzer, the mouse model for Usher syndrome type 1D

12 August 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=6028713

The varieties of auditory neuropathy.

12 August 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=6028713

Unconventional myosins and the genetics of hearing loss

12 August 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=6028713

Gene transfer into the mammalian inner ear using HSV-1 and vaccinia virus vectors

12 August 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=6028713

Clinical features of the prevalent form of childhood deafness, DFNB1, due to a connexin-26 gene defect: implications for genetic counselling

12 August 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=6028713

Mutations in the gene encoding B1 subunit of H+-ATPase cause renal tubular acidosis with sensorineural deafness

12 August 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=6028713

Mutations in the human alpha-tectorin gene cause autosomal dominant non-syndromic hearing impairment

12 August 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=6028713

Adenoviral-mediated gene transfer into guinea pig cochlear cells in vivo

12 August 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=6028713

Genetic epidemiological studies of early-onset deafness in the U.S. school-age population

12 August 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=6028713

Ongoing proliferation of melanocytes in the stria vascularis of adult guinea pigs

12 August 2018

1 reference

PubMed

12 December 2020

Genetic epidemiological studies of congenital/prelingual deafness in Turkey: Population structure and mating type are major determinants of mutation identification

1 reference

PubMed

12 December 2020

Mitochondrial deafness

1 reference

PubMed

12 December 2020

Identifiers

DOI

10.3389/FNMOL.2018.00221

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:29997477%20AND%20SRC:MED&resulttype=core&format=json

7 June 2020

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:29997477%20AND%20SRC:MED&resulttype=core&format=json

7 June 2020

PubMed publication ID

29997477

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:29997477%20AND%20SRC:MED&resulttype=core&format=json