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2 r cells and strial marginal cells but not to stereociliary ankle links or pillar cells, which nonspec
3 FL-) whirlin in photoreceptors and hair cell stereociliary bases is important for the USH type 2 prot
4 2+ extrusion by pumps, Ca2+ binding to fixed stereociliary buffers, and Ca2+ binding to mobile buffer
7 the molecular mechanism underlying cochlear stereociliary bundle development and hearing loss pathog
10 mmalian Phenotype Level 4 abnormal hair cell stereociliary bundle morphology and related phenotypes;
13 The vertebrate hair cell is named for its stereociliary bundle or hair bundle that protrudes from
14 and guidance, hair follicle orientation, and stereociliary bundle orientation in inner ear sensory ha
15 es Frzb in regulating cochlear extension and stereociliary bundle orientation in vitro, and that Wnt5
16 By contrast, vinculin planar asymmetry and stereociliary bundle orientation were restored in Fz3(-/
17 e homology, disrupts neural tube closure and stereociliary bundle orientation, and shows genetic inte
19 ey must result from an active process in the stereociliary bundle suggested to underlie amplification
20 receptor potential are the deflection of the stereociliary bundle, and the subsequent flow of transdu
21 lanar cell polarity and morphogenesis of the stereociliary bundle, including bundle fragmentation or
22 n, several aspects of the development of the stereociliary bundle, including its elongation and orien
23 lar their mechanotransduction organelle, the stereociliary bundle, requires highly organized remodeli
24 is involved both in the morphogenesis of the stereociliary bundle, the sensory antenna of inner ear h
30 he ankle link complex (ALC) at the hair cell stereociliary bundle; however, little is known about the
31 air cells that fail to properly orient their stereociliary bundles along the mediolateral axis of the
33 ially differentiated hair cells fail to form stereociliary bundles and degenerate by apoptosis in the
34 t mechanism that actively reorients auditory stereociliary bundles and reveals an unexpected role of
35 polarity is necessary for normal hearing as stereociliary bundles are only sensitive to vibrations i
36 ed of neuromasts, patches of hair cells with stereociliary bundles arranged with morphological mirror
37 require a coordinated alignment of hair cell stereociliary bundles as an essential element of mechano
39 eated water-jet stimulation of the hair cell stereociliary bundles caused adaptation of the action po
40 yonic development, hair cells acquire apical stereociliary bundles for mechanosensation, basolateral
41 gnificant disruptions in the polarization of stereociliary bundles in mouse cochlea as a result of de
44 ts for spontaneous oscillations of hair cell stereociliary bundles in the lower vertebrate inner ear.
45 nolabelling demonstrated TMC1 throughout the stereociliary bundles in wild type but not in Lhfpl5 mut
48 The morphogenesis and maintenance of these stereociliary bundles is a tightly regulated process req
50 ration are mediated through the vibration of stereociliary bundles located on the lumenal surfaces of
51 tube closure, as well as the orientation of stereociliary bundles of sensory hair cells in the inner
52 cture of the cochlea that is attached to the stereociliary bundles of the outer hair cells (OHCs), el
53 in vertebrates is the uniform orientation of stereociliary bundles of the sensory hair cells in the m
55 ransduction, sound-induced vibrations of the stereociliary bundles on the sensory hair cells are conv
56 on, sound-evoked vibrations of the hair cell stereociliary bundles open mechanotransducer (MET) ion c
57 ), with oppositely oriented planar-polarized stereociliary bundles that detect motion in opposite dir
58 two groups of cells with oppositely oriented stereociliary bundles that meet at a line of polarity re
59 trical signals depends upon mechanosensitive stereociliary bundles that project from the apical surfa
60 ends on mechanosensitive ion channels in the stereociliary bundles that project from the apical surfa
61 itory hair cells requires highly specialized stereociliary bundles that project from their apical sur
62 ral conductances and synaptic properties yet stereociliary bundles were absent, or small and nonfunct
66 s the architecture and mechanosensitivity of stereociliary bundles, improves hearing thresholds, and
67 CP defects, including mis-oriented hair cell stereociliary bundles, in Bbs8 and Ift20 single mutants
68 ells showed normal development of individual stereociliary bundles, indicating that asymmetry was est
69 be, and misorientation of cochlear hair cell stereociliary bundles, indicative of defects in planar c
70 mbrane directly overlies the inner hair cell stereociliary bundles, these data provide the most accur
81 rmally may be stimulated by the reduction in stereociliary Ca2+ when gating springs rupture and trans
83 ractions in the glycocalyx contribute to the stereociliary coherence that is essential for hearing.
84 RV1 signaling in vitro, are localized to the stereociliary compartments that overlap with AC6 distrib
85 d can elicit tonic OHC motility in mice with stereociliary defects that eliminate cochlear amplificat
87 ir-cell bundle in vivo is required to ensure stereociliary displacement similarity, increasing the sp
91 ncing identified a downregulation of several stereociliary genes in the Myo7a-deficient cochlea, indi
92 apillary electrophoresis, we showed that the stereociliary glycocalyx acts as a negatively charged po
93 es for the ALC in regulating inner hair cell stereociliary growth and differentiation as well as oute
94 isoforms are required for normal vestibular stereociliary growth, although they may play slightly di
95 , including the shape and orientation of the stereociliary hair bundle essential for sound detection.
96 the Trpml3 gene cause disorganization of the stereociliary hair bundle, structural aberrations in out
99 ouse embryos displayed disrupted polarity of stereociliary hair cells in the cochlea, a characteristi
101 uency region, rootlet length correlates with stereociliary height but between regions it changes disp
102 hair bundle cohesiveness and the absence of stereociliary imprints in the TM observed in these mice
106 ressed in the vestibular organs, where their stereociliary localizations are similar to those of deve
107 e acoustic trauma-induced tip link damage or stereociliary loss by disrupting tip links or ablating t
108 HCs that express characteristic synaptic and stereociliary markers and survive to adulthood, although
109 e hair cell, a detachment of the apical, non-stereociliary membrane of the hair cell from the underly
117 ured the coherence and phase of the relative stereociliary motions with a dual-beam differential inte
121 osis at P6 and argue for connections between stereociliary PMCA2 density, hair cell apoptosis, and de
122 ations do not impact on the cation selective stereociliary process or the endolymphatic potential, ou
123 ian vertebrates amplification is produced by stereociliary processes, related to the mechanotransduce
124 PDZ-binding site with the PDZ1 domain of the stereociliary protein harmonin, and potentially via a we
126 found that BAIAP2L2 interacts with other key stereociliary proteins involved in normal hair bundle mo
127 Instead, the interaction of myosin-1c with stereociliary receptors depended on its calmodulin-bindi
128 d differentiation as well as outer hair cell stereociliary rigidity and organization during developme
130 -VI in cuticular plates and association with stereociliary rootlets suggest that this isozyme partici
132 the stereociliary taper, peaked in the lower stereociliary shaft, and declined progressively toward t
133 losed that radixin labeling commenced in the stereociliary taper, peaked in the lower stereociliary s
134 hat the cdh23 mutation may be harmful to the stereociliary tip link and cause the hair cell apoptosis
135 on: cadherin 23 (Cdh23), a candidate for the stereociliary tip link, and phosphatidylinositol 4,5-bis
136 pre-embedding EM immunogold microscopy, with stereociliary tip-link and subcuticular plate sites.
138 Myo1c that mediates CaM-sensitive binding to stereociliary tips and to PIP2 immobilized on a solid su
141 L- and C-terminal (C-) whirlins in hair cell stereociliary tips participate in stereociliary elongati
142 filaments, myosin-Ibeta is found mostly near stereociliary tips, myosin-VI is largely absent, and myo
143 ously that Myo1c interacts with molecules at stereociliary tips, the site of transduction, through se
146 maller number of channels transported to the stereociliary tips; this may stem from impaired TMC1 bin