Genetics of auditory mechano-electrical transduction

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Genetics of auditory mechano-electrical transduction is …
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scholarly articleQ13442814
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P6179Dimensions Publication ID1011562704
P356DOI10.1007/S00424-014-1552-9
P932PMC publication ID4281357
P698PubMed publication ID24957570
P5875ResearchGate publication ID263397578

P50authorChristine PetitQ510206
Nicolas MichalskiQ57419574
P2860cites workEPS8, encoding an actin-binding protein of cochlear hair cell stereocilia, is a new causal gene for autosomal recessive profound deafnessQ21202842
KCNQ4, a novel potassium channel expressed in sensory outer hair cells, is mutated in dominant deafnessQ22008780
A defect in harmonin, a PDZ domain-containing protein expressed in the inner ear sensory hair cells, underlies Usher syndrome type 1CQ24290185
Mutation of CDH23, encoding a new member of the cadherin gene family, causes Usher syndrome type 1DQ24290695
Mutations of the protocadherin gene PCDH15 cause Usher syndrome type 1FQ24291312
Mutations in TBC1D24, a gene associated with epilepsy, also cause nonsyndromic deafness DFNB86.Q37441862
Correlation of actin crosslinker and capper expression levels with stereocilia growth phasesQ37563927
One-step generation of mice carrying reporter and conditional alleles by CRISPR/Cas-mediated genome engineeringQ37651840
Transduction without tip links in cochlear hair cells is mediated by ion channels with permeation properties distinct from those of the mechano-electrical transducer channel.Q37705498
Planar cell polarity in the inner ear.Q38058904
Revisiting planar cell polarity in the inner ear.Q38096733
Auditory distortions: origins and functionsQ38153676
The responses of inner and outer hair cells in the basal turn of the guinea-pig cochlea and in the mouse cochlea grown in vitroQ38592435
Loss of K-Cl co-transporter KCC3 causes deafness, neurodegeneration and reduced seizure thresholdQ39962346
Cadherin 23 is a component of the tip link in hair-cell stereociliaQ40571247
Harmonin-b, an actin-binding scaffold protein, is involved in the adaptation of mechanoelectrical transduction by sensory hair cellsQ42060669
Correction of deafness in shaker-2 mice by an unconventional myosin in a BAC transgene.Q42455765
A large-conductance calcium-selective mechanotransducer channel in mammalian cochlear hair cellsQ79301431
The tip-link antigen, a protein associated with the transduction complex of sensory hair cells, is protocadherin-15Q79810543
Vlgr1 is required for proper stereocilia maturation of cochlear hair cellsQ28588493
Mutations in Cdh23, encoding a new type of cadherin, cause stereocilia disorganization in waltzer, the mouse model for Usher syndrome type 1DQ28589238
The mouse Ames waltzer hearing-loss mutant is caused by mutation of Pcdh15, a novel protocadherin geneQ28589565
The very large G-protein-coupled receptor VLGR1: a component of the ankle link complex required for the normal development of auditory hair bundles.Q28589815
Defects in whirlin, a PDZ domain molecule involved in stereocilia elongation, cause deafness in the whirler mouse and families with DFNB31Q28590107
A mouse model for human deafness DFNB22 reveals that hearing impairment is due to a loss of inner hair cell stimulation.Q28590148
Cthrc1 selectively activates the planar cell polarity pathway of Wnt signaling by stabilizing the Wnt-receptor complexQ28590249
Regulation of PCDH15 function in mechanosensory hair cells by alternative splicing of the cytoplasmic domainQ28590789
Myosin XVa and whirlin, two deafness gene products required for hair bundle growth, are located at the stereocilia tips and interact directlyQ28590821
Physical and functional interaction between protocadherin 15 and myosin VIIa in mechanosensory hair cellsQ28592402
Mutations in a new scaffold protein Sans cause deafness in Jackson shaker miceQ28592537
Interactions in the network of Usher syndrome type 1 proteinsQ28592874
Photoreceptor expression of the Usher syndrome type 1 protein protocadherin 15 (USH1F) and its interaction with the scaffold protein harmonin (USH1C)Q28593040
Identification of Vangl2 and Scrb1 as planar polarity genes in mammalsQ28593071
The role of Frizzled3 and Frizzled6 in neural tube closure and in the planar polarity of inner-ear sensory hair cellsQ28593180
ATP8B1 is essential for maintaining normal hearingQ28593592
A Receptor-Like Inositol Lipid Phosphatase Is Required for the Maturation of Developing Cochlear Hair BundlesQ28593796
TMHS is an integral component of the mechanotransduction machinery of cochlear hair cellsQ28593852
Myosin-XVa is required for tip localization of whirlin and differential elongation of hair-cell stereociliaQ28593953
Mice with altered KCNQ4 K+ channels implicate sensory outer hair cells in human progressive deafnessQ28594332
An unusually powerful mode of low-frequency sound interference due to defective hair bundles of the auditory outer hair cellsQ28594961
Mutations in Cdh23 cause nonsyndromic hearing loss in waltzer miceQ28595024
Stereocilin-deficient mice reveal the origin of cochlear waveform distortionsQ28595046
One-step generation of mice carrying mutations in multiple genes by CRISPR/Cas-mediated genome engineeringQ29547524
Sharpened cochlear tuning in a mouse with a genetically modified tectorial membraneQ30048524
TMC function in hair cell transductionQ30413040
Molecular architecture of the chick vestibular hair bundleQ30417485
PTK7 regulates myosin II activity to orient planar polarity in the mammalian auditory epitheliumQ30418906
Kif3a regulates planar polarization of auditory hair cells through both ciliary and non-ciliary mechanismsQ30425548
Fast adaptation in vestibular hair cells requires myosin-1c activityQ30437793
Three deaf mice: mouse models for TECTA-based human hereditary deafness reveal domain-specific structural phenotypes in the tectorial membraneQ30439995
β-Actin and fascin-2 cooperate to maintain stereocilia lengthQ30447400
Developmental changes in the cochlear hair cell mechanotransducer channel and their regulation by transmembrane channel-like proteinsQ30452945
Mutations in the novel protocadherin PCDH15 cause Usher syndrome type 1FQ24291535
A functional study of plasma-membrane calcium-pump isoform 2 mutants causing digenic deafnessQ24293479
Carcinoembryonic antigen-related cell adhesion molecule 16 interacts with alpha-tectorin and is mutated in autosomal dominant hearing loss (DFNA4).Q24294638
Usher syndrome type I G (USH1G) is caused by mutations in the gene encoding SANS, a protein that associates with the USH1C protein, harmoninQ24294773
From flies' eyes to our ears: mutations in a human class III myosin cause progressive nonsyndromic hearing loss DFNB30Q24298272
Alterations of the CIB2 calcium- and integrin-binding protein cause Usher syndrome type 1J and nonsyndromic deafness DFNB48.Q24299865
The DFNB31 gene product whirlin connects to the Usher protein network in the cochlea and retina by direct association with USH2A and VLGR1Q24301988
A new compartment at stereocilia tips defined by spatial and temporal patterns of myosin IIIa expressionQ24305683
Mutations in the VLGR1 gene implicate G-protein signaling in the pathogenesis of Usher syndrome type IIQ24306120
PDZD7 is a modifier of retinal disease and a contributor to digenic Usher syndromeQ24309592
Mutations in LOXHD1, an evolutionarily conserved stereociliary protein, disrupt hair cell function in mice and cause progressive hearing loss in humansQ24313238
Defective myosin VIIA gene responsible for Usher syndrome type 1BQ24314638
Regulation of planar cell polarity by Smurf ubiquitin ligasesQ24317525
A novel mutation in the potassium channel gene KVLQT1 causes the Jervell and Lange-Nielsen cardioauditory syndromeQ24318498
Characterization of unconventional MYO6, the human homologue of the gene responsible for deafness in Snell's waltzer miceQ24323049
Nonsyndromic deafness DFNA1 associated with mutation of a human homolog of the Drosophila gene diaphanousQ24324252
Twinfilin 2 regulates actin filament lengths in cochlear stereociliaQ24324361
A novel gene for Usher syndrome type 2: mutations in the long isoform of whirlin are associated with retinitis pigmentosa and sensorineural hearing lossQ24337039
Myosin VIIa, harmonin and cadherin 23, three Usher I gene products that cooperate to shape the sensory hair cell bundleQ24337137
Mutations in a novel gene with transmembrane domains underlie Usher syndrome type 3.Q24533348
Usher syndrome 1D and nonsyndromic autosomal recessive deafness DFNB12 are caused by allelic mutations of the novel cadherin-like gene CDH23Q24535604
A mutation of beta -actin that alters depolymerization dynamics is associated with autosomal dominant developmental malformations, deafness, and dystoniaQ24546532
Usher syndrome type III: revised genomic structure of the USH3 gene and identification of novel mutationsQ24563482
Vezatin, a novel transmembrane protein, bridges myosin VIIA to the cadherin-catenins complexQ24597349
TMC1 and TMC2 are components of the mechanotransduction channel in hair cells of the mammalian inner earQ24599460
Multi-isotope imaging mass spectrometry reveals slow protein turnover in hair-cell stereociliaQ24601390
The deaf jerker mouse has a mutation in the gene encoding the espin actin-bundling proteins of hair cell stereocilia and lacks espins.Q24603825
Efficient genome editing in zebrafish using a CRISPR-Cas systemQ24610828
Disruption of Mks1 localization to the mother centriole causes cilia defects and developmental malformations in Meckel-Gruber syndromeQ24622660
Mechanotransduction in mouse inner ear hair cells requires transmembrane channel-like genesQ24634356
Localization of inner hair cell mechanotransducer channels using high-speed calcium imaging.Q24647513
Mutations of ESPN cause autosomal recessive deafness and vestibular dysfunctionQ24675125
An actin molecular treadmill and myosins maintain stereocilia functional architecture and self-renewalQ24676711
Eps8 regulates hair bundle length and functional maturation of mammalian auditory hair cellsQ27321482
The structure of the harmonin/sans complex reveals an unexpected interaction mode of the two Usher syndrome proteinsQ27659663
Structure of MyTH4-FERM domains in myosin VIIa tail bound to cargoQ27666702
Connexin 26 mutations in hereditary non-syndromic sensorineural deafnessQ28115871
Deafness and imbalance associated with inactivation of the secretory Na-K-2Cl co-transporterQ28137646
A deletion mutation in GJB6 cooperating with a GJB2 mutation in trans in non-syndromic deafness: A novel founder mutation in Ashkenazi JewsQ28201634
Dominant and recessive deafness caused by mutations of a novel gene, TMC1, required for cochlear hair-cell functionQ28202014
A large deletion including most of GJB6 in recessive non syndromic deafness: a digenic effect?Q28207515
A deletion involving the connexin 30 gene in nonsyndromic hearing impairmentQ28216378
Connexin30 (Gjb6)-deficiency causes severe hearing impairment and lack of endocochlear potentialQ28218302
A mutation in the gamma actin 1 (ACTG1) gene causes autosomal dominant hearing loss (DFNA20/26)Q28235113
IsK and KvLQT1: mutation in either of the two subunits of the slow component of the delayed rectifier potassium channel can cause Jervell and Lange-Nielsen syndromeQ28251067
KCNE1 mutations cause jervell and Lange-Nielsen syndromeQ28253362
Hearing is normal without connexin30Q28283001
Mutations of the RDX gene cause nonsyndromic hearing loss at the DFNB24 locusQ28283551
Connexin30-mediated intercellular communication plays an essential role in epithelial repair in the cochleaQ28285901
Polypeptide composition of the mammalian tectorial membraneQ28300248
Coordinated control of connexin 26 and connexin 30 at the regulatory and functional level in the inner earQ28302365
The novel mouse mutation Oblivion inactivates the PMCA2 pump and causes progressive hearing lossQ28473896
Mammalian Otolin: a multimeric glycoprotein specific to the inner ear that interacts with otoconial matrix protein Otoconin-90 and Cerebellin-1Q28475514
Regulation of stereocilia length by myosin XVa and whirlin depends on the actin-regulatory protein Eps8Q28504869
Myosin IIIB uses an actin-binding motif in its espin-1 cargo to reach the tips of actin protrusionsQ28505690
Disruption of Bardet-Biedl syndrome ciliary proteins perturbs planar cell polarity in vertebratesQ28506652
Distinct subdomain organization and molecular composition of a tight junction with adherens junction featuresQ28506754
Mouse models of USH1C and DFNB18: phenotypic and molecular analyses of two new spontaneous mutations of the Ush1c geneQ28506758
Characterization of a Dchs1 mutant mouse reveals requirements for Dchs1-Fat4 signaling during mammalian developmentQ28507073
Ultrastructural findings in the inner ear of Jackson shaker miceQ28507373
Deletion of PDZD7 disrupts the Usher syndrome type 2 protein complex in cochlear hair cells and causes hearing loss in miceQ28507622
Regulation of polarized extension and planar cell polarity in the cochlea by the vertebrate PCP pathwayQ28509023
Usherin, the defective protein in Usher syndrome type IIA, is likely to be a component of interstereocilia ankle links in the inner ear sensory cellsQ28509442
Shaker-1 mutations reveal roles for myosin VIIA in both development and function of cochlear hair cellsQ28509764
Stereocilin connects outer hair cell stereocilia to one another and to the tectorial membraneQ28510238
Whirlin complexes with p55 at the stereocilia tip during hair cell development.Q28510548
Murine dishevelled 3 functions in redundant pathways with dishevelled 1 and 2 in normal cardiac outflow tract, cochlea, and neural tube developmentQ28511382
The mouse tectorins. Modular matrix proteins of the inner ear homologous to components of the sperm-egg adhesion systemQ28511714
A core cochlear phenotype in USH1 mouse mutants implicates fibrous links of the hair bundle in its cohesion, orientation and differential growthQ28511852
A type VII myosin encoded by the mouse deafness gene shaker-1Q28512902
The mouse Snell's waltzer deafness gene encodes an unconventional myosin required for structural integrity of inner ear hair cellsQ28513409
Molecular characterization of the ankle-link complex in cochlear hair cells and its role in the hair bundle functioningQ28513848
An alteration in ELMOD3, an Arl2 GTPase-activating protein, is associated with hearing impairment in humansQ28536871
Alström Syndrome protein ALMS1 localizes to basal bodies of cochlear hair cells and regulates cilium-dependent planar cell polarityQ28567235
Myosin VIIa and sans localization at stereocilia upper tip-link density implicates these Usher syndrome proteins in mechanotransductionQ28569812
Cadherin 23 is a component of the transient lateral links in the developing hair bundles of cochlear sensory cellsQ28575837
The chloride intracellular channel protein CLIC5 is expressed at high levels in hair cell stereocilia and is essential for normal inner ear functionQ28578311
The concentrations of calcium buffering proteins in mammalian cochlear hair cellsQ28582823
Sec24b selectively sorts Vangl2 to regulate planar cell polarity during neural tube closureQ28584955
Role of myosin VI in the differentiation of cochlear hair cellsQ28585164
The novel mouse mutant, chuzhoi, has disruption of Ptk7 protein and exhibits defects in neural tube, heart and lung development and abnormal planar cell polarity in the ear.Q28585297
Targeted disruption of the Kvlqt1 gene causes deafness and gastric hyperplasia in miceQ28585784
Ciliary proteins link basal body polarization to planar cell polarity regulationQ28585995
Reduced climbing and increased slipping adaptation in cochlear hair cells of mice with Myo7a mutationsQ28586011
A mouse model for human hearing loss DFNB30 due to loss of function of myosin IIIAQ28586478
Mutations in a new gene encoding a protein of the hair bundle cause non-syndromic deafness at the DFNB16 locusQ28587113
PTK7/CCK-4 is a novel regulator of planar cell polarity in vertebratesQ28587777
Otogelin: a glycoprotein specific to the acellular membranes of the inner earQ28588114
Loss of mammal-specific tectorial membrane component carcinoembryonic antigen cell adhesion molecule 16 (CEACAM16) leads to hearing impairment at low and high frequenciesQ30453291
Localization of Usher 1 proteins to the photoreceptor calyceal processes, which are absent from miceQ30455456
Diaphanous homolog 3 (Diap3) overexpression causes progressive hearing loss and inner hair cell defects in a transgenic mouse model of human deafnessQ30457932
Vezatin, an integral membrane protein of adherens junctions, is required for the sound resilience of cochlear hair cellsQ30463528
Mutations of the mouse ELMO domain containing 1 gene (Elmod1) link small GTPase signaling to actin cytoskeleton dynamics in hair cell stereociliaQ30467882
Usher type 1G protein sans is a critical component of the tip-link complex, a structure controlling actin polymerization in stereociliaQ30475800
Roles of the espin actin-bundling proteins in the morphogenesis and stabilization of hair cell stereocilia revealed in CBA/CaJ congenic jerker miceQ30476139
Genomic duplication and overexpression of TJP2/ZO-2 leads to altered expression of apoptosis genes in progressive nonsyndromic hearing loss DFNA51.Q30477623
The dimensions and composition of stereociliary rootlets in mammalian cochlear hair cells: comparison between high- and low-frequency cells and evidence for a connection to the lateral membraneQ30478611
Restoration of connexin26 protein level in the cochlea completely rescues hearing in a mouse model of human connexin30-linked deafnessQ30478884
Hair bundles are specialized for ATP delivery via creatine kinase.Q30479206
Connexin30 deficiency causes instrastrial fluid-blood barrier disruption within the cochlear stria vascularisQ30479250
Cadherin-23, myosin VIIa and harmonin, encoded by Usher syndrome type I genes, form a ternary complex and interact with membrane phospholipidsQ30479852
Gelsolin plays a role in the actin polymerization complex of hair cell stereociliaQ30480367
Mosaic complementation demonstrates a regulatory role for myosin VIIa in actin dynamics of stereociliaQ30481360
Harmonin mutations cause mechanotransduction defects in cochlear hair cells.Q30488014
Fast adaptation and Ca2+ sensitivity of the mechanotransducer require myosin-XVa in inner but not outer cochlear hair cellsQ30488481
In vivo and in vitro effects of two novel gamma-actin (ACTG1) mutations that cause DFNA20/26 hearing impairmentQ30489007
Myosin IIIa boosts elongation of stereocilia by transporting espin 1 to the plus ends of actin filamentsQ30490501
Actin-bundling protein TRIOBP forms resilient rootlets of hair cell stereocilia essential for hearingQ30494750
Radixin deficiency causes deafness associated with progressive degeneration of cochlear stereociliaQ30496716
Sensitivity, polarity, and conductance change in the response of vertebrate hair cells to controlled mechanical stimuliQ30500976
Progressive hearing loss and gradual deterioration of sensory hair bundles in the ears of mice lacking the actin-binding protein Eps8L2.Q30543591
Targeted ablation of connexin26 in the inner ear epithelial gap junction network causes hearing impairment and cell deathQ30578611
β-actin and γ-actin are each dispensable for auditory hair cell development but required for Stereocilia maintenanceQ30990855
MAGI-1, a candidate stereociliary scaffolding protein, associates with the tip-link component cadherin 23.Q33381195
A claudin-9-based ion permeability barrier is essential for hearing.Q33495556
MyosinVIIa interacts with Twinfilin-2 at the tips of mechanosensory stereocilia in the inner ear.Q33506085
Gipc1 has a dual role in Vangl2 trafficking and hair bundle integrity in the inner ear.Q33793330
Mutations in the gamma-actin gene (ACTG1) are associated with dominant progressive deafness (DFNA20/26).Q33905602
A recessive contiguous gene deletion causing infantile hyperinsulinism, enteropathy and deafness identifies the Usher type 1C geneQ33916587
Adaptation in hair cells.Q33938585
Whole exome sequencing and homozygosity mapping identify mutation in the cell polarity protein GPSM2 as the cause of nonsyndromic hearing loss DFNB82.Q33960515
Claudin 14 knockout mice, a model for autosomal recessive deafness DFNB29, are deaf due to cochlear hair cell degenerationQ33967701
Cross-links between stereocilia in the guinea pig organ of Corti, and their possible relation to sensory transductionQ42457296
Ionic currents of outer hair cells isolated from the guinea-pig cochleaQ42460882
Dynamical control of the shape and size of stereocilia and microvilliQ42468948
Bbs8, together with the planar cell polarity protein Vangl2, is required to establish left-right asymmetry in zebrafish.Q42474570
Electrophysiological measurements of the stria vascularis potentials in vivoQ42487624
KCNJ10 (Kir4.1) potassium channel knockout abolishes endocochlear potentialQ42515610
Probing the pore of the auditory hair cell mechanotransducer channel in turtleQ42628739
A novel deletion involving the connexin-30 gene, del(GJB6-d13s1854), found in trans with mutations in the GJB2 gene (connexin-26) in subjects with DFNB1 non-syndromic hearing impairment.Q43073195
Reorganization of actin during repair of hair bundle mechanoreceptorsQ44174651
A targeted deletion in alpha-tectorin reveals that the tectorial membrane is required for the gain and timing of cochlear feedback.Q44342057
Receptor-mediated endocytosis of a chemoreceptor involved in triggering the discharge of cnidae in a sea anemone tentacleQ44573065
An active motor model for adaptation by vertebrate hair cellsQ44614827
The actions of calcium on the mechano-electrical transducer current of turtle hair cellsQ44730740
A dominant mutation in the stereocilia-expressing gene TBC1D24 is a probable cause for nonsyndromic hearing impairmentQ44775711
A chemical-genetic strategy implicates myosin-1c in adaptation by hair cells.Q45712448
Development and properties of stereociliary link types in hair cells of the mouse cochleaQ46388853
Mechanotransduction: the elusive hair cell transduction channel revealed?Q46439374
Response characteristics of mammalian cochlear hair cellsQ46608590
Dynamic compartmentalization of protein tyrosine phosphatase receptor Q at the proximal end of stereocilia: implication of myosin VI-based transportQ46645141
Primary cilium migration depends on G-protein signalling control of subapical cytoskeleton.Q46854784
Mutations in cadherin 23 affect tip links in zebrafish sensory hair cellsQ47074176
Biallelic nonsense mutations in the otogelin-like gene (OTOGL) in a child affected by mild to moderate hearing impairmentQ48014054
Evidence for multiple, developmentally regulated isoforms of Ptprq on hair cells of the inner ear.Q50436970
The digenic hypothesis unraveled: the GJB6 del(GJB6-D13S1830) mutation causes allele-specific loss of GJB2 expression in cis.Q50442870
Specific loss of connexin 26 expression in ductal sweat gland epithelium associated with the deletion mutation del(GJB6-D13S1830).Q50470202
The transduction channel filter in auditory hair cells.Q50470934
Twister mutant mice are defective for otogelin, a component specific to inner ear acellular membranes.Q50493046
A new locus for late-onset, progressive, hereditary hearing loss DFNA20 maps to 17q25.Q50498025
Mutation of the Na-K-Cl co-transporter gene Slc12a2 results in deafness in mice.Q50500527
Effects of noise on cochlear potentials and endolymph potassium concentration recorded with potassium-selective electrodesQ50608923
Loss of Fat4 disrupts PCP signaling and oriented cell division and leads to cystic kidney disease.Q51953017
Sorting of actin isoforms in chicken auditory hair cells.Q52195984
Mechano-electrical transducer currents in hair cells of the cultured neonatal mouse cochleaQ52415919
Kinetics of the receptor current in bullfrog saccular hair cells.Q52706206
A molecular blueprint at the apical surface establishes planar asymmetry in cochlear hair cells.Q53086214
Composition and supramolecular organization of the tectorial membrane.Q53879857
Positive endocochlear potential: mechanism of production by marginal cells of stria vascularis.Q53883513
The mechanical properties of chick (Gallus domesticus) sensory hair bundles: relative contributions of structures sensitive to calcium chelation and subtilisin treatment.Q53892048
Cross-links between stereocilia in the guinea pig cochlea.Q53900443
The extent of adaptation in bullfrog saccular hair cells.Q54022790
A quantitative comparison of mechanoelectrical transduction in vestibular and auditory hair cells of neonatal mice.Q55380927
Ablation of connexin30 in transgenic mice alters expression patterns of connexin26 and connexin32 in glial cells and leptomeningesQ58493621
Rapid renewal of auditory hair bundlesQ59074461
The CD2 isoform of protocadherin-15 is an essential component of the tip-link complex in mature auditory hair cellsQ33991492
Maintained expression of the planar cell polarity molecule Vangl2 and reformation of hair cell orientation in the regenerating inner ear.Q34037450
Usher syndrome: from genetics to pathogenesis.Q34101060
Deafness and renal tubular acidosis in mice lacking the K-Cl co-transporter Kcc4.Q34125574
USH3A transcripts encode clarin-1, a four-transmembrane-domain protein with a possible role in sensory synapsesQ34134919
Mechanical relaxation of the hair bundle mediates adaptation in mechanoelectrical transduction by the bullfrog's saccular hair cellQ34180933
Mechanisms of endocochlear potential generation by stria vascularisQ34185692
Ionic basis of the receptor potential in a vertebrate hair cellQ34211370
Extracellular current flow and the site of transduction by vertebrate hair cellsQ34252990
Inner ear defects induced by null mutation of the isk geneQ34412325
TBC1D24 mutation causes autosomal-dominant nonsyndromic hearing loss.Q34415041
A deafness mutation isolates a second role for the tectorial membrane in hearingQ34431118
Mechano-electrical transduction currents in isolated vestibular hair cells of the chickQ34469243
Mutation of a gene encoding a protein with extracellular matrix motifs in Usher syndrome type IIa.Q34471433
Tip-link integrity and mechanical transduction in vertebrate hair cellsQ34654174
Cadherin 23 and protocadherin 15 interact to form tip-link filaments in sensory hair cellsQ34680349
Prestin-driven cochlear amplification is not limited by the outer hair cell membrane time constantQ35128559
Coupling of the mechanotransduction machinery and F-actin polymerization in the cochlear hair bundlesQ35534680
KCNQ4, a K+ channel mutated in a form of dominant deafness, is expressed in the inner ear and the central auditory pathway.Q35682356
CLIC5 stabilizes membrane-actin filament linkages at the base of hair cell stereocilia in a molecular complex with radixin, taperin, and myosin VI.Q35795161
GPSM2 mutations cause the brain malformations and hearing loss in Chudley-McCullough syndromeQ36017073
NHERF family and NHE3 regulationQ36132944
THE VISUAL CELLS AND VISUAL PIGMENT OF THE MUDPUPPY, NECTURUSQ36187611
The organization of actin filaments in the stereocilia of cochlear hair cellsQ36201573
Preliminary biochemical characterization of the stereocilia and cuticular plate of hair cells of the chick cochleaQ36221707
Mutations in OTOGL, encoding the inner ear protein otogelin-like, cause moderate sensorineural hearing loss.Q36365707
Localization of PDZD7 to the stereocilia ankle-link associates this scaffolding protein with the Usher syndrome protein networkQ36456242
Bardet-Biedl syndrome.Q36466434
Genetic interaction between members of the Vangl family causes neural tube defects in miceQ36491169
Genome editing with RNA-guided Cas9 nuclease in zebrafish embryosQ36739960
Mutations of KCNJ10 together with mutations of SLC26A4 cause digenic nonsyndromic hearing loss associated with enlarged vestibular aqueduct syndromeQ37189151
Who needs tip links? Backwards transduction by hair cellsQ37268766
The role of transmembrane channel-like proteins in the operation of hair cell mechanotransducer channelsQ37268781
P275copyright licenseCreative Commons Attribution 4.0 InternationalQ20007257
P433issue1
P921main subjectcellQ7868
mechanotransductionQ11936292
enzymeQ8047
membrane proteinQ423042
nervous systemQ9404
sensory nervous systemQ11101
macromolecular substanceQ75174158
physiological phenomenonQ66615932
P304page(s)49-72
P577publication date2014-06-25
2015-01-01
P1433published inPfluegers ArchivQ1091689
P1476titleGenetics of auditory mechano-electrical transduction
P478volume467

Reverse relations

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