ライフサイエンスコーパス: stereociliary

1 The stereociliary actin-bundling protein espin was targeted

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

5 The asymmetric location of stereociliary bundle (hair bundle) on the apical surface

6 Complex assembly likely occurs at the stereociliary bundle but not along the protein transport

7 the molecular mechanism underlying cochlear stereociliary bundle development and hearing loss pathog

8 Defects in this complex cause stereociliary bundle disorganization and hearing loss.

9 he ankle link region of the mechanosensitive stereociliary bundle in hair cells.

10 toxin-damaged utricles and the maturation of stereociliary bundle morphology.

11 Vestibular identity was based on: (1) stereociliary bundle morphology; (2) spacing of hair cel

12 The vertebrate hair cell is named for its stereociliary bundle or hair bundle that protrudes from

13 and guidance, hair follicle orientation, and stereociliary bundle orientation in inner ear sensory ha

14 es Frzb in regulating cochlear extension and stereociliary bundle orientation in vitro, and that Wnt5

15 By contrast, vinculin planar asymmetry and stereociliary bundle orientation were restored in Fz3(-/

16 e homology, disrupts neural tube closure and stereociliary bundle orientation, and shows genetic inte

17 regulates the development of unidirectional stereociliary bundle orientation.

18 ey must result from an active process in the stereociliary bundle suggested to underlie amplification

19 receptor potential are the deflection of the stereociliary bundle, and the subsequent flow of transdu

20 lanar cell polarity and morphogenesis of the stereociliary bundle, including bundle fragmentation or

21 n, several aspects of the development of the stereociliary bundle, including its elongation and orien

22 lar their mechanotransduction organelle, the stereociliary bundle, requires highly organized remodeli

23 is involved both in the morphogenesis of the stereociliary bundle, the sensory antenna of inner ear h

24 evident in the polarized organization of the stereociliary bundle.

25 nesis of the auditory sensory epithelium and stereociliary bundle.

26 tops of the hair cell stereocilia within the stereociliary bundle.

27 ractions and is required for cohesion of the stereociliary bundle.

28 n mechanically sensitive ion channels in the stereociliary bundle.

29 he ankle link complex (ALC) at the hair cell stereociliary bundle; however, little is known about the

30 air cells that fail to properly orient their stereociliary bundles along the mediolateral axis of the

31 ially differentiated hair cells fail to form stereociliary bundles and degenerate by apoptosis in the

32 t mechanism that actively reorients auditory stereociliary bundles and reveals an unexpected role of

33 polarity is necessary for normal hearing as stereociliary bundles are only sensitive to vibrations i

34 ed of neuromasts, patches of hair cells with stereociliary bundles arranged with morphological mirror

35 require a coordinated alignment of hair cell stereociliary bundles as an essential element of mechano

36 showing that even severe dysmorphogenesis of stereociliary bundles can be corrected.

37 gnificant disruptions in the polarization of stereociliary bundles in mouse cochlea as a result of de

38 The observed disorganized stereociliary bundles in the bpck inner ear and the conv

39 Otoliths, which are connected to stereociliary bundles in the inner ear, serve as inertia

40 ts for spontaneous oscillations of hair cell stereociliary bundles in the lower vertebrate inner ear.

41 nolabelling demonstrated TMC1 throughout the stereociliary bundles in wild type but not in Lhfpl5 mut

42 t mechanical deflections of their actin-rich stereociliary bundles into electrochemical signals.

43 In the mammalian cochlea, stereociliary bundles located on mechanosensory hair cel

44 ration are mediated through the vibration of stereociliary bundles located on the lumenal surfaces of

45 tube closure, as well as the orientation of stereociliary bundles of sensory hair cells in the inner

46 in vertebrates is the uniform orientation of stereociliary bundles of the sensory hair cells in the m

47 The structures of stereociliary bundles of trans-differentiated hair cells

48 ransduction, sound-induced vibrations of the stereociliary bundles on the sensory hair cells are conv

49 ends on mechanosensitive ion channels in the stereociliary bundles that project from the apical surfa

50 is the uniform orientation of the hair cell stereociliary bundles within the cochlea.

51 nctions in hair cell survival, maturation of stereociliary bundles, and auditory function.

52 The induced hair cells display stereociliary bundles, attract neuronal processes and ex

53 s the architecture and mechanosensitivity of stereociliary bundles, improves hearing thresholds, and

54 CP defects, including mis-oriented hair cell stereociliary bundles, in Bbs8 and Ift20 single mutants

55 ells showed normal development of individual stereociliary bundles, indicating that asymmetry was est

56 be, and misorientation of cochlear hair cell stereociliary bundles, indicative of defects in planar c

57 mbrane directly overlies the inner hair cell stereociliary bundles, these data provide the most accur

58 a role in the polarization of the developing stereociliary bundles.

59 olarized membrane protrusions reminiscent of stereociliary bundles.

60 lusters displaying hair cell-like cells with stereociliary bundles.

61 es in the shape and orientation of hair cell stereociliary bundles.

62 eased during the formation and maturation of stereociliary bundles.

63 , neural tube defects and disrupted cochlear stereociliary bundles.

64 We also developed a model of stereociliary Ca2+ homeostasis that incorporates four re

65 rmally may be stimulated by the reduction in stereociliary Ca2+ when gating springs rupture and trans

66 r cells, where it is regulated by changes in stereociliary calcium.

67 ractions in the glycocalyx contribute to the stereociliary coherence that is essential for hearing.

68 Stereociliary development is unaffected in samba mice, b

69 hair cell stereociliary tips participate in stereociliary elongation.

70 apillary electrophoresis, we showed that the stereociliary glycocalyx acts as a negatively charged po

71 es for the ALC in regulating inner hair cell stereociliary growth and differentiation as well as oute

72 isoforms are required for normal vestibular stereociliary growth, although they may play slightly di

73 the Trpml3 gene cause disorganization of the stereociliary hair bundle, structural aberrations in out

74 ouse embryos displayed disrupted polarity of stereociliary hair cells in the cochlea, a characteristi

75 mechanically sensitive ion channels in their stereociliary (hair) bundle.

76 uency region, rootlet length correlates with stereociliary height but between regions it changes disp

77 The stereociliary link protein cadherin 23 (Cdh23) was targe

78 This stereociliary localization domain was absent in Va(J) he

79 Here we refined the stereociliary localization of TRPML3 and investigated co

80 ressed in the vestibular organs, where their stereociliary localizations are similar to those of deve

81 e acoustic trauma-induced tip link damage or stereociliary loss by disrupting tip links or ablating t

82 HCs that express characteristic synaptic and stereociliary markers and survive to adulthood, although

83 e hair cell, a detachment of the apical, non-stereociliary membrane of the hair cell from the underly

84 density (UTLD), where CDH23 inserts into the stereociliary membrane.

85 achment between glycan components of apposed stereociliary membranes.

86 protein despite the presence of PIP2 in the stereociliary membranes.

87 Stereociliary morphological defects in USH2 mutant mice

88 Hair-cell fate and stereociliary morphology and function were examined usin

89 fication, two mechanisms have been proposed: stereociliary motility and somatic motility.

90 lative squeezing but not the sliding mode of stereociliary motion.

91 ured the coherence and phase of the relative stereociliary motions with a dual-beam differential inte

92 g vesicular trafficking, cell migration, and stereociliary movements of hair cells.

93 ations do not impact on the cation selective stereociliary process or the endolymphatic potential, ou

94 ian vertebrates amplification is produced by stereociliary processes, related to the mechanotransduce

95 PDZ-binding site with the PDZ1 domain of the stereociliary protein harmonin, and potentially via a we

96 Instead, the interaction of myosin-1c with stereociliary receptors depended on its calmodulin-bindi

97 d differentiation as well as outer hair cell stereociliary rigidity and organization during developme

98 Moreover, pejvakin localizes to stereociliary rootlets in hair cells and is required for

99 -VI in cuticular plates and association with stereociliary rootlets suggest that this isozyme partici

100 ly and indirectly, via microtubules, to some stereociliary rootlets.

101 the stereociliary taper, peaked in the lower stereociliary shaft, and declined progressively toward t

102 losed that radixin labeling commenced in the stereociliary taper, peaked in the lower stereociliary s

103 hat the cdh23 mutation may be harmful to the stereociliary tip link and cause the hair cell apoptosis

104 on: cadherin 23 (Cdh23), a candidate for the stereociliary tip link, and phosphatidylinositol 4,5-bis

105 pre-embedding EM immunogold microscopy, with stereociliary tip-link and subcuticular plate sites.

106 A unique coupling between HCN1 and stereociliary tip-link protein protocadherin 15 has been

107 Myo1c that mediates CaM-sensitive binding to stereociliary tips and to PIP2 immobilized on a solid su

108 Binding of Myo1c to stereociliary tips of cochlear and vestibular hair cells

109 ied multiprotein complex associated with the stereociliary tips of hair bundles.

110 L- and C-terminal (C-) whirlins in hair cell stereociliary tips participate in stereociliary elongati

111 filaments, myosin-Ibeta is found mostly near stereociliary tips, myosin-VI is largely absent, and myo

112 ously that Myo1c interacts with molecules at stereociliary tips, the site of transduction, through se

113 f radial links and an increase in tenting of stereociliary tips.

114 l membrane attachment crowns are seen at the stereociliary tips.