(Q37087936)

18 August 2017

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title

Quiet as a mouse: dissecting the molecular and genetic basis of hearing (English)

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18 August 2017

Steve D M Brown

1

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18 August 2017

Rachel E Hardisty-Hughes

series ordinal

2

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18 August 2017

Philomena Mburu

series ordinal

3

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18 August 2017

publication date

19 February 2008

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18 August 2017

published in

Nature Reviews Genetics

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18 August 2017

volume

9

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18 August 2017

issue

4

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18 August 2017

page(s)

277-290

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Connexins in hearing loss: a comprehensive overview

18 August 2017

cites work

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Mutation analysis of the mouse myosin VIIA deafness gene

21 January 2018

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21 January 2018

1 reference

31 March 2020

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Focusing on the genetics of hearing: you ain't heard nothin' yet.

31 March 2020

1 reference

Development and properties of stereociliary link types in hair cells of the mouse cochlea

31 March 2020

1 reference

Mutation of Celsr1 disrupts planar polarity of inner ear hair cells and causes severe neural tube defects in the mouse

31 March 2020

1 reference

Identification of Vangl2 and Scrb1 as planar polarity genes in mammals

31 March 2020

1 reference

A type VII myosin encoded by the mouse deafness gene shaker-1

31 March 2020

1 reference

The mouse Snell's waltzer deafness gene encodes an unconventional myosin required for structural integrity of inner ear hair cells

31 March 2020

1 reference

Correction of deafness in shaker-2 mice by an unconventional myosin in a BAC transgene.

31 March 2020

1 reference

Defects in whirlin, a PDZ domain molecule involved in stereocilia elongation, cause deafness in the whirler mouse and families with DFNB31

31 March 2020

1 reference

The mouse Ames waltzer hearing-loss mutant is caused by mutation of Pcdh15, a novel protocadherin gene

31 March 2020

1 reference

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

31 March 2020

1 reference

A systematic, genome-wide, phenotype-driven mutagenesis programme for gene function studies in the mouse

31 March 2020

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Genome-wide, large-scale production of mutant mice by ENU mutagenesis.

31 March 2020

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Identification of 17 hearing impaired mouse strains in the TMGC ENU-mutagenesis screen.

31 March 2020

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A forward genetics screen in mice identifies recessive deafness traits and reveals that pejvakin is essential for outer hair cell function

31 March 2020

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TRPA1 is a candidate for the mechanosensitive transduction channel of vertebrate hair cells

31 March 2020

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TRPA1 contributes to cold, mechanical, and chemical nociception but is not essential for hair-cell transduction

31 March 2020

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

31 March 2020

1 reference

31 March 2020

A Mouse for All Reasons

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An actin molecular treadmill and myosins maintain stereocilia functional architecture and self-renewal

31 March 2020

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The deaf jerker mouse has a mutation in the gene encoding the espin actin-bundling proteins of hair cell stereocilia and lacks espins.

31 March 2020

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Balanced levels of Espin are critical for stereociliary growth and length maintenance.

31 March 2020

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Myosin XVa localizes to the tips of inner ear sensory cell stereocilia and is essential for staircase formation of the hair bundle

31 March 2020

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Myosin-XVa is required for tip localization of whirlin and differential elongation of hair-cell stereocilia

31 March 2020

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Myosin XVa and whirlin, two deafness gene products required for hair bundle growth, are located at the stereocilia tips and interact directly

31 March 2020

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Mutant analysis reveals whirlin as a dynamic organizer in the growing hair cell stereocilium

31 March 2020

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The deaf mouse mutant whirler suggests a role for whirlin in actin filament dynamics and stereocilia development

31 March 2020

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Whirlin complexes with p55 at the stereocilia tip during hair cell development.

31 March 2020

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MPP1 links the Usher protein network and the Crumbs protein complex in the retina

31 March 2020

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Drosophila Stardust is a partner of Crumbs in the control of epithelial cell polarity

31 March 2020

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Identification of the protein 4.1 binding interface on glycophorin C and p55, a homologue of the Drosophila discs-large tumor suppressor protein

31 March 2020

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CASK and protein 4.1 support F-actin nucleation on neurexins

31 March 2020

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CIP98, a novel PDZ domain protein, is expressed in the central nervous system and interacts with calmodulin-dependent serine kinase

31 March 2020

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A novel gene for Usher syndrome type 2: mutations in the long isoform of whirlin are associated with retinitis pigmentosa and sensorineural hearing loss

31 March 2020

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Mouse models of USH1C and DFNB18: phenotypic and molecular analyses of two new spontaneous mutations of the Ush1c gene

31 March 2020

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Usher syndrome: molecular links of pathogenesis, proteins and pathways

31 March 2020

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Usher syndrome: from genetics to pathogenesis.

31 March 2020

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The very large G-protein-coupled receptor VLGR1: a component of the ankle link complex required for the normal development of auditory hair bundles.

31 March 2020

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Vlgr1 is required for proper stereocilia maturation of cochlear hair cells

31 March 2020

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The Mass1frings mutation underlies early onset hearing impairment in BUB/BnJ mice, a model for the auditory pathology of Usher syndrome IIC.

31 March 2020

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Usherin is required for maintenance of retinal photoreceptors and normal development of cochlear hair cells

31 March 2020

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USH3A transcripts encode clarin-1, a four-transmembrane-domain protein with a possible role in sensory synapses

31 March 2020

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A defect in harmonin, a PDZ domain-containing protein expressed in the inner ear sensory hair cells, underlies Usher syndrome type 1C

31 March 2020

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Myosin VIIa, harmonin and cadherin 23, three Usher I gene products that cooperate to shape the sensory hair cell bundle

31 March 2020

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The Usher syndrome proteins cadherin 23 and harmonin form a complex by means of PDZ-domain interactions

31 March 2020

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The DFNB31 gene product whirlin connects to the Usher protein network in the cochlea and retina by direct association with USH2A and VLGR1

31 March 2020

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Scaffold protein harmonin (USH1C) provides molecular links between Usher syndrome type 1 and type 2

31 March 2020

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Interactions in the network of Usher syndrome type 1 proteins

31 March 2020

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Usherin, the defective protein in Usher syndrome type IIA, is likely to be a component of interstereocilia ankle links in the inner ear sensory cells

31 March 2020

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Physical and functional interaction between protocadherin 15 and myosin VIIa in mechanosensory hair cells

31 March 2020

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Molecular characterization of the ankle-link complex in cochlear hair cells and its role in the hair bundle functioning

31 March 2020

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Cadherin 23 is a component of the transient lateral links in the developing hair bundles of cochlear sensory cells

31 March 2020

1 reference

Mutations in cadherin 23 affect tip links in zebrafish sensory hair cells

31 March 2020

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Cadherin 23 is a component of the tip link in hair-cell stereocilia

31 March 2020

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Cadherin 23 and protocadherin 15 interact to form tip-link filaments in sensory hair cells

31 March 2020

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The tip-link antigen, a protein associated with the transduction complex of sensory hair cells, is protocadherin-15

31 March 2020

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PCDH15 is expressed in the neurosensory epithelium of the eye and ear and mutant alleles are responsible for both USH1F and DFNB23

31 March 2020

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Usher I syndrome: unravelling the mechanisms that underlie the cohesion of the growing hair bundle in inner ear sensory cells

31 March 2020

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A Receptor-Like Inositol Lipid Phosphatase Is Required for the Maturation of Developing Cochlear Hair Bundles

31 March 2020

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Contributions of mouse models to understanding of age- and noise-related hearing loss

31 March 2020

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Strain background effects and genetic modifiers of hearing in mice

31 March 2020

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A major gene affecting age-related hearing loss is common to at least ten inbred strains of mice

31 March 2020

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Association of cadherin 23 with polygenic inheritance and genetic modification of sensorineural hearing loss

31 March 2020

1 reference

mdfw:A Deafness Susceptibility Locus That Interacts with Deaf Waddler (dfw)

31 March 2020

1 reference

Dominant and recessive deafness caused by mutations of a novel gene, TMC1, required for cochlear hair-cell function

31 March 2020

1 reference

Beethoven, a mouse model for dominant, progressive hearing loss DFNA36.

31 March 2020

1 reference

Multiple quantitative trait loci modify cochlear hair cell degeneration in the Beethoven (Tmc1Bth) mouse model of progressive hearing loss DFNA36.

31 March 2020

1 reference

The aetiology of otitis media with effusion: a review

31 March 2020

1 reference

Genetic susceptibility to otitis media

31 March 2020

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Mouse models as a tool to unravel the genetic basis for human otitis media

31 March 2020

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A mutation in the F-box gene, Fbxo11, causes otitis media in the Jeff mouse

31 March 2020

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Mutation at the Evi1 locus in Junbo mice causes susceptibility to otitis media

31 March 2020

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Association of the FBXO11 gene with chronic otitis media with effusion and recurrent otitis media: the Minnesota COME/ROM Family Study

31 March 2020

1 reference

Identification and analysis of functional elements in 1% of the human genome by the ENCODE pilot project

31 March 2020

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MicroRNA gene expression in the mouse inner ear.

31 March 2020

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

31 March 2020

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Fast adaptation in vestibular hair cells requires myosin-1c activity

31 March 2020

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Prestin-based outer hair cell electromotility in knockin mice does not appear to adjust the operating point of a cilia-based amplifier

31 March 2020

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A deafness mutation isolates a second role for the tectorial membrane in hearing

31 March 2020

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Stereocilia defects in waltzer (Cdh23), shaker1 (Myo7a) and double waltzer/shaker1 mutant mice

31 March 2020

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Myo15 function is distinct from Myo6, Myo7a and pirouette genes in development of cochlear stereocilia

31 March 2020

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A new spontaneous mutation in the mouse Ames waltzer gene, Pcdh15.

31 March 2020

1 reference

Mutations in a plasma membrane Ca2+-ATPase gene cause deafness in deafwaddler mice

31 March 2020

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A new spontaneous mouse mutation in the Kcne1 gene

31 March 2020

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Twister mutant mice are defective for otogelin, a component specific to inner ear acellular membranes

31 March 2020

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Transcription profiling of inner ears from Pou4f3(ddl/ddl) identifies Gfi1 as a target of the Pou4f3 deafness gene

31 March 2020

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Multiple mutations in mouse Chd7 provide models for CHARGE syndrome

31 March 2020

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The homeobox gene Emx2 underlies middle ear and inner ear defects in the deaf mouse mutant pardon

31 March 2020

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The Notch ligand Jagged1 is required for inner ear sensory development.

31 March 2020

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The mouse slalom mutant demonstrates a role for Jagged1 in neuroepithelial patterning in the organ of Corti

31 March 2020

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Balance and hearing deficits in mice with a null mutation in the gene encoding plasma membrane Ca2+-ATPase isoform 2

31 March 2020

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Selective cochlear degeneration in mice lacking the F-box protein, Fbx2, a glycoprotein-specific ubiquitin ligase subunit.

31 March 2020

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Skeletal overgrowth and deafness in mice lacking fibroblast growth factor receptor 3.

31 March 2020

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An Fgf8 mutant allelic series generated by Cre- and Flp-mediated recombination

31 March 2020

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Embryonic lethality and vascular defects in mice lacking the Notch ligand Jagged1

31 March 2020

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Notch signalling pathway mediates hair cell development in mammalian cochlea

31 March 2020

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Inner ear defects induced by null mutation of the isk gene

31 March 2020

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Targeted disruption of otog results in deafness and severe imbalance

31 March 2020

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Essential role of POU–domain factor Brn-3c in auditory and vestibular hair cell development

31 March 2020

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Targeted disruption of mouse Pds provides insight about the inner-ear defects encountered in Pendred syndrome

31 March 2020

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31 March 2020

Identifiers

DOI

10.1038/NRG2309

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18 August 2017

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