A core cochlear phenotype in USH1 mouse mutants implicates fibrous links of the hair bundle in its cohesion, orientation and differential growth
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Integrating the biophysical and molecular mechanisms of auditory hair cell mechanotransductionClarin-1, encoded by the Usher Syndrome III causative gene, forms a membranous microdomain: possible role of clarin-1 in organizing the actin cytoskeletonReview series: The cell biology of hearingCadherins and mechanotransduction by hair cellsCytoskeleton Molecular Motors: Structures and Their Functions in NeuronSensing sound: molecules that orchestrate mechanotransduction by hair cellsStructures of usher syndrome 1 proteins and their complexesClass III myosins shape the auditory hair bundles by limiting microvilli and stereocilia growth.Cadherin 23-C Regulates Microtubule Networks by Modifying CAMSAP3's Function.The structure of the harmonin/sans complex reveals an unexpected interaction mode of the two Usher syndrome proteinsStructure of MyTH4-FERM domains in myosin VIIa tail bound to cargoHarmonin (Ush1c) is required in zebrafish Müller glial cells for photoreceptor synaptic development and functionUsher syndrome IIIA gene clarin-1 is essential for hair cell function and associated neural activationStereocilin connects outer hair cell stereocilia to one another and to the tectorial membrane.Localization and expression of clarin-1, the Clrn1 gene product, in auditory hair cells and photoreceptorsMyosin VIIa and sans localization at stereocilia upper tip-link density implicates these Usher syndrome proteins in mechanotransductionRegulation of PCDH15 function in mechanosensory hair cells by alternative splicing of the cytoplasmic domainThe small GTPase Rac1 regulates auditory hair cell morphogenesis.TMHS is an integral component of the mechanotransduction machinery of cochlear hair cellsDevelopmental regulation of planar cell polarity and hair-bundle morphogenesis in auditory hair cells: lessons from human and mouse genetics.Usher syndrome: Hearing loss, retinal degeneration and associated abnormalities.Zebrafish Models for the Mechanosensory Hair Cell Dysfunction in Usher Syndrome 3 Reveal That Clarin-1 Is an Essential Hair Bundle Protein.The physiology of mechanoelectrical transduction channels in hearing.Genetics of auditory mechano-electrical transduction.Kif3a regulates planar polarization of auditory hair cells through both ciliary and non-ciliary mechanisms.Compound heterozygosity of the functionally null Cdh23(v-ngt) and hypomorphic Cdh23(ahl) alleles leads to early-onset progressive hearing loss in mice.A mouse model for nonsyndromic deafness (DFNB12) links hearing loss to defects in tip links of mechanosensory hair cells.Rescue of hearing and vestibular function by antisense oligonucleotides in a mouse model of human deafness.Digenic inheritance of deafness caused by 8J allele of myosin-VIIA and mutations in other Usher I genesLocalization of Usher 1 proteins to the photoreceptor calyceal processes, which are absent from mice.A new mouse mutant of the Cdh23 gene with early-onset hearing loss facilitates evaluation of otoprotection drugs.Mutations of the mouse ELMO domain containing 1 gene (Elmod1) link small GTPase signaling to actin cytoskeleton dynamics in hair cell stereocilia.Linking genes underlying deafness to hair-bundle development and function.Usher type 1G protein sans is a critical component of the tip-link complex, a structure controlling actin polymerization in stereocilia.Deafness and retinal degeneration in a novel USH1C knock-in mouse modelThe novel PMCA2 pump mutation Tommy impairs cytosolic calcium clearance in hair cells and links to deafness in mice.Cadherin-23, myosin VIIa and harmonin, encoded by Usher syndrome type I genes, form a ternary complex and interact with membrane phospholipids.Cadherins as targets for genetic diseases.Harmonin mutations cause mechanotransduction defects in cochlear hair cells.Ush1c gene expression levels in the ear and eye suggest different roles for Ush1c in neurosensory organs in a new Ush1c knockout mouse
P2860
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P2860
A core cochlear phenotype in USH1 mouse mutants implicates fibrous links of the hair bundle in its cohesion, orientation and differential growth
description
2008 թուականի Ապրիլին հրատարակուած գիտական յօդուած
@hyw
2008 թվականի ապրիլին հրատարակված գիտական հոդված
@hy
artículu científicu espublizáu en 2008
@ast
im April 2008 veröffentlichter wissenschaftlicher Artikel
@de
scientific journal article
@en
vedecký článok (publikovaný 2008/04/01)
@sk
vědecký článek publikovaný v roce 2008
@cs
wetenschappelijk artikel (gepubliceerd op 2008/04/01)
@nl
наукова стаття, опублікована у квітні 2008
@uk
مقالة علمية (نشرت في أبريل 2008)
@ar
name
A core cochlear phenotype in U ...... tation and differential growth
@ast
A core cochlear phenotype in U ...... tation and differential growth
@en
A core cochlear phenotype in U ...... tation and differential growth
@nl
type
label
A core cochlear phenotype in U ...... tation and differential growth
@ast
A core cochlear phenotype in U ...... tation and differential growth
@en
A core cochlear phenotype in U ...... tation and differential growth
@nl
prefLabel
A core cochlear phenotype in U ...... tation and differential growth
@ast
A core cochlear phenotype in U ...... tation and differential growth
@en
A core cochlear phenotype in U ...... tation and differential growth
@nl
P2093
P921
P3181
P356
P1433
P1476
A core cochlear phenotype in U ...... tation and differential growth
@en
P2093
Dominique Weil
Emilie Bizard
Gaelle Lefèvre
Léa Lepelletier
Uwe Wolfrum
Vincent Michel
P304
P3181
P356
10.1242/DEV.012922
P407
P577
2008-04-01T00:00:00Z