ライフサイエンスコーパス: stereocilia

1 y multiple rows of actin-filled projections (stereocilia).

2 y the tension of 'tip links' interconnecting stereocilia.

3 ent damage to some surviving outer hair cell stereocilia.

4 led protein and EGFP-beta-actin into nascent stereocilia.

5 is dispensable for the initial formation of stereocilia.

6 hat sets a blueprint for the location of the stereocilia.

7 le and also to cochlear hair cell nuclei and stereocilia.

8 tein cargos within developing mechanosensory stereocilia.

9 undle's rootlets from the actin cores of the stereocilia.

10 uperfamily, also immunolocalizd to hair cell stereocilia.

11 USH1G and PCDH15 may form another complex in stereocilia.

12 let structure for anchoring the tip links of stereocilia.

13 0%) is accompanied by the loss and fusion of stereocilia.

14 progressive degeneration of outer hair cell stereocilia.

15 the actin cytoskeleton dynamics of hair cell stereocilia.

16 ical membrane forms appropriately around the stereocilia.

17 en the liquid within the hair bundle and the stereocilia.

18 f cation channels near the tips of hair cell stereocilia.

19 ll microvilli, the topological equivalent of stereocilia.

20 t sound through deflection of mechanosensory stereocilia.

21 or the growth and function of microvilli and stereocilia.

22 ard stereocilia tips of the bundle's longest stereocilia.

23 e fragments bound near the tips of hair cell stereocilia.

24 expression in the taper region of hair cell stereocilia.

25 les formed by espin, an actin crosslinker in stereocilia.

26 ts in abnormally thin and slightly shortened stereocilia.

27 essed along the membrane of mature hair cell stereocilia.

28 springs, the tip links, connecting adjacent stereocilia.

29 and targeting of the protein to the tips of stereocilia.

30 Myo7a motors and coordinate force sensing in stereocilia.

31 ing on tip-link proteins connecting adjacent stereocilia.

32 mechanosensitive hair bundles formed by the stereocilia.

33 ibrations measured near the mechanosensitive stereocilia.

34 YO3A cargo protein endogenously expressed in stereocilia [2], MYO3B targets and carries ESPN1 to COS7

35 e results in the fusion of the hair bundle's stereocilia, a resorption of the parallel actin bundles

36 the elongation and differential identity of stereocilia across rows.

37 tin-regulatory element for elongation of the stereocilia actin core.

38 Altogether, the data support a model whereby stereocilia actin cores are largely static, with dynamic

39 ively, our analyses support a model in which stereocilia actin cores are stable structures that incor

40 ese models, we perform an unbiased survey of stereocilia actin dynamics in more than 500 utricle hair

41 Stereocilia actin filaments are uniformly oriented with

42 Stereocilia actin incorporation is remarkably slow and r

43 Here we assessed stereocilia actin turnover by monitoring incorporation o

44 otransducer (MT) channels at the tips of the stereocilia, activated by tension in interciliary tip li

45 Loss and fusion of stereocilia also occurs in the striolar region of the ut

46 posed of several rows of regularly organized stereocilia and a kinocilium, is essential for mechanotr

47 ithin stable actin-based protrusions such as stereocilia and calycal processes.

48 s tip links that interconnect mechanosensory stereocilia and convey force to yet unidentified transdu

49 ar filaments that connect pairs of hair cell stereocilia and convey tension to mechanosensitive chann

50 lly observed distributions of Myosin IIIa in stereocilia and filopodia.

51 ytoskeletal processes, including microvilli, stereocilia and filopodia.

52 earing and the transduction mechanism in the stereocilia and for melanosome transport in the retina,

53 chlear hair cells revealed loss of some tall stereocilia and gaps in the v-shaped bundle, although ti

54 nse, displayed highly disorganized hair-cell stereocilia and had no detectable MYO7A protein.

55 sion of MyoXVa results in both elongation of stereocilia and increased accumulation of Eps8 at stereo

56 on prevented normal elongation of vestibular stereocilia and irregularly widened them.

57 rminal differentiation manifests as immature stereocilia and kinocilia on the apical surface of hair

58 s to extracellular linkages that connect the stereocilia and kinocilium into a bundle and regulate it

59 nd rigidity for normal mechanosensitivity of stereocilia and may contribute to resilient cytoskeletal

60 Cadherin linkages between adjacent stereocilia and microvilli are essential for mechanotran

61 and adaptor proteins: Myo7a/SANS/Harmonin in stereocilia and Myo7b/ANKS4B/Harmonin in microvilli.

62 s; they unexpectedly limit the elongation of stereocilia and of subsequently regressing microvilli, t

63 e for plastin 1 in the preservation of adult stereocilia and optimal hearing.

64 erminal domain traffics to these specialized stereocilia and prevents disassembly of their actin core

65 s elongations and fusions of inner hair cell stereocilia and progressive degeneration of outer hair c

66 RhoC co-localizes with Fam65b in stereocilia and regulates Fam65b oligomerization.

67 are regenerated, and regenerated HCs display stereocilia and synapses.

68 gether is >1000 times the pivot stiffness of stereocilia and that these links can slide in the plane

69 s such as the side surfaces of the hair cell stereocilia and the intercalated disks of isolated cardi

70 r bundles move, the viscous friction between stereocilia and the surrounding liquid poses a fundament

71 product localizes to the tips of the tallest stereocilia and the tectorial membrane (TM).

72 a stiffness and the gap size between the IHC stereocilia and the tectorial membrane determine the cha

73 proximately 200 nm nanodomain at the tips of stereocilia and this localization requires the presence

74 as well as in the connection between the OHC stereocilia and TM, a linkage essential for mechanical a

75 at the point of attachment of the TM to the stereocilia and, when mutated, results in ADNSHL at the

76 ate between functionally distinct classes of stereocilia, and are independently required to assemble

77 tin-filled projections such as filopodia and stereocilia, and directional migration.

78 easure mechanical properties of nodal cilia, stereocilia, and motile cilia-anatomically similar struc

79 ansduction channel is expressed in hair cell stereocilia, and previous studies show that its activity

80 the nine Usher-associated genes have splayed stereocilia, and some show delayed maturation of ribbon

81 uired for long-term maintenance of hair cell stereocilia, and that its dysfunction causes hearing los

82 d GPR98 have been reported to form hair cell stereocilia ankle-links, harmonin localizes to the stere

83 l-autonomous role of pejvakin in maintaining stereocilia architecture that is critical for hair cell

84 Stereocilia are actin-based protrusions on auditory sens

85 Tip links between adjacent stereocilia are believed to gate mechano-electrical tran

86 Some mechanotransducing shorter row stereocilia are missing, whereas the remaining ones exhi

87 Since stereocilia are primarily composed of crosslinked, paral

88 Yet, how the lengths of actin-based stereocilia are regulated remains poorly understood.

89 Extracellular tip links that interconnect stereocilia are thought to gate mechanosensitive channel

90 MET channels, located at the tops of stereocilia, are poised to detect tension induced by hai

91 the tectorial membrane is detached from OHC stereocilia, arguing that the tuning of radial vibration

92 try to show that PDZD7 is expressed in chick stereocilia at a comparable molecular abundance to GPR98

93 o a highly dissipative serial arrangement of stereocilia at distortion frequencies, precluding their

94 One membrane domain, including the stereocilia basal tapers and approximately 1 mum of the

95 3(343A) allele disrupts the structure of the stereocilia bundle and affects long-term function of aud

96 ed an inner hair-cell and an outer hair-cell stereocilia bundle and simulated the effect of probe sti

97 The stereocilia bundle is the mechano-transduction apparatus

98 ng frequency, the active motility of the IHC stereocilia bundle reduced the power dissipation in the

99 nated by the viscous friction around the IHC stereocilia bundle--the IHC stereocilia increased the ST

100 fluid flow around the inner hair cell (IHC) stereocilia bundle.

101 placodes and give rise to hair cells bearing stereocilia bundles and a kinocilium.

102 ack of PDZD7 leads to the disorganization of stereocilia bundles and a reduction in mechanotransducti

103 Stereocilia bundles are partially disorganized, disorien

104 In the mammalian cochlea, the stereocilia bundles are situated in the subtectorial spa

105 t the ectopic hair cells display specialized stereocilia bundles similar to endogenous hair cells.

106 numerous microvilli or stereocilia, ungraded stereocilia bundles, and bundle rounding and closure.

107 the small isoform remarkably develop normal stereocilia bundles.

108 2 is essential for hearing and localizes to stereocilia, but its exact function is unknown.

109 HCN2 protein is immunolocalized to hair-cell stereocilia by both z-stack confocal and pre-embedding E

110 ultrastructural alterations of the hair cell stereocilia by mirroring them on the tectorial membrane.

111 It is not clear how stereocilia can stick together laterally but still shear

112 MyoXVa and that mice lacking Eps8 show short stereocilia compared to MyoXVa- and whirlin-deficient mi

113 w HCs resided in the outer HC region, formed stereocilia, contained mechanoelectrical transduction ch

114 e arrays of mechanosensitive microvilli-like stereocilia crowning the auditory hair cells, is essenti

115 croscopy analyses of hair cells demonstrated stereocilia degeneration in these mice.

116 lop, but mechanotransduction is affected and stereocilia deteriorate.

117 a potential role in endosomal recycling and stereocilia development/maintenance, and the basolateral

118 FSCN2 expression increases when these stereocilia differentially elongate, suggesting that FSC

119 at Atoh1 plays a crucial role to initiate HC stereocilia differentiation independently of HC viabilit

120 s and vestibular impairment characterized by stereocilia disorganization, hair cell loss, and endococ

121 d pressure levels, often it takes >500 nm of stereocilia displacement to saturate hair-cell mechano-t

122 d magnitude of the forces between individual stereocilia during small hair-bundle deflections.

123 finding connecting ELMOD1 deficiencies with stereocilia dysmorphologies thus establishes a link betw

124 Among these are the stereocilia, each with a core of several hundred actin f

125 We find that the close apposition of stereocilia effectively immobilizes the liquid between t

126 ne lifted away from the cuticular plate, and stereocilia elongated and fused.

127 mouse orthologue of GPSM2 affects actin-rich stereocilia elongation in auditory and vestibular hair c

128 8 is essential for the initial elongation of stereocilia, Eps8L2 is required for their maintenance in

129 the tectorial membrane stimulates hair-cell stereocilia evenly, probes deflect stereocilia unevenly.

130 ecombination, neither actin isoform left the stereocilia, except at the tips.

131 Conflicting models propose that stereocilia F-actin cores are either continually renewed

132 vident in overt force sensors at the tips of stereocilia for vertebrate hearing and the touch recepto

133 actin-associated proteins are essential for stereocilia formation and maintenance, and their absence

134 only in apical regions, but abnormalities of stereocilia formation were present throughout the cochle

135 sed expression of myosin VIIa and failure of stereocilia formation.

136 nd by force-sensing photomicrometry that the stereocilia formed elastic connections with one another

137 tions of the molecular motor myosin 15 stunt stereocilia growth and cause deafness.

138 Actin turnover in stereocilia has previously been studied by transfecting

139 kidney and intestinal microvilli, inner ear stereocilia, immune synapses, endocytic patches, adhesio

140 Analysis of the stereocilia imprint pattern at the undersurface of the t

141 r the proper organization and maintenance of stereocilia in auditory hair cells.

142 in photoreceptors and the ankle link of the stereocilia in hair cells.

143 the rootlet component TRIOBP at the base of stereocilia in injectoporated hair cells, a pattern that

144 to be downregulated along the length of the stereocilia in maturing Myo6 mutant mice.

145 that Eps8 fails to accumulate at the tips of stereocilia in the absence of MyoXVa, that overexpressio

146 ether with shortening of a defined subset of stereocilia in the hair cell bundle.

147 subunits, and is targeted to the tips of the stereocilia in the sensory hair bundle, where the MET ch

148 Although the hair-cell stereocilia in vivo deflect <100 nm even at high sound p

149 In contrast, turnover in chick stereocilia in vivo is much slower.

150 C-1 is colocalized with F-actin in hair cell stereocilia in vivo, using a hemagglutinin-tagged PC-1 K

151 ation in the STS because of the presence IHC stereocilia increased as the stimulating frequency decre

152 n around the IHC stereocilia bundle--the IHC stereocilia increased the STS power dissipation by 50- t

153 Capzb-null stereocilia initially developed normally but later short

154 volved in limiting the growth of transducing stereocilia.Inner ear hair cells detect sound through de

155 In addition, stereocilia integrity may hinge on immobilizing actin, w

156 The precise assembly of inner ear hair cell stereocilia into rows of increasing height is critical f

157 orption of the parallel actin bundles of the stereocilia into the cytoplasm of the hair cell, a detac

158 The maintenance of sensory hair cell stereocilia is critical for lifelong hearing; however, m

159 The precise architecture of hair cell stereocilia is essential for normal hearing.

160 ology; the sequence of structural changes in stereocilia is known, and a modest number of proteins ma

161 Because dimension control of the inner ear's stereocilia is particularly precise, we studied the CAPZ

162 the most abundant actin-bundling proteins of stereocilia is plastin 1, but its function has never bee

163 ike (ESPNL), primarily present in developing stereocilia, is also a myosin-III cargo and is essential

164 In contrast to stereocilia, kinocilia are not critical for hair-cell me

165 nking protein fascin-2 cooperate to maintain stereocilia length and auditory function.

166 ly, MYO3A and MYO3B, are thought to regulate stereocilia length by transporting cargos that control a

167 diate actin filament severing, contribute to stereocilia length maintenance.

168 actin dynamics are essential for controlling stereocilia length.

169 lymerization at stereocilia tips to maintain stereocilia length.

170 ow these proteins work together to influence stereocilia length.

171 air cells to establish the required range of stereocilia lengths within a single cell.

172 4 hours, also suggesting rapid regulation of stereocilia lengths.

173 s (myo7a, calretinin, parvalbumin, myo6) and stereocilia-like structures expressing F-actin and espin

174 ize simultaneously immuno-gold particles and stereocilia links, both of only a few nanometers in diam

175 y rootlets in hair cells and is required for stereocilia maintenance and mechanosensory function of t

176 Stereocilia maintenance is essential because auditory ha

177 protein has a different function in a common stereocilia maintenance pathway.

178 rimental evidence for the dynamic control of stereocilia morphology by the mechanotransduction curren

179 All stereocilia moved by approximately the same angular defl

180 Although stereocilia moved coherently and in phase at the stimulu

181 Dawley) of either sex, PIP2 localizes within stereocilia, near stereocilia tips.

182 Thus, rapid turnover in stereocilia occurs only at the tips and not by a treadmi

183 ls are gated by tip links, which connect the stereocilia of a hair cell in the direction of their mec

184 NXA5 in bundles and none were upregulated in stereocilia of Anxa5(-/-) mice.

185 cation channels located near the tips of the stereocilia of auditory and vestibular inner ear hair ce

186 dle protein that is localized at the tips of stereocilia of both cochlear and vestibular hair cells.

187 Although stereocilia of both inner and outer hair cells of Myo15(

188 Stereocilia of Fam65b-deficient murine hair cells start

189 They are located in the stereocilia of hair cells and opened by the tension in s

190 e components into the mechanically sensitive stereocilia of hair cells.

191 ein localizes to the plasma membranes of the stereocilia of inner and outer hair cells of the inner e

192 ice, CIB2 is localized to the mechanosensory stereocilia of inner ear hair cells and to retinal photo

193 notransducer channels at the tips of sensory stereocilia of inner ear hair cells are gated by the ten

194 in Pls1 KO, but in young adult animals, the stereocilia of inner hair cells were reduced in width an

195 he basal taper of the mechanically sensitive stereocilia of murine hair cells.

196 The stereocilia of OHCs were comparatively less affected; ho

197 We show that Np55 is expressed in stereocilia of outer hair cells (OHCs) but not inner hai

198 In St3gal5(-/-) mice, stereocilia of outer hair cells showed signs of degenera

199 cytoskeletal architecture in the developing stereocilia of sensory hair cells.

200 Stereocilia of St3gal5(-/-) inner hair cells were fused

201 with immunolocalization of CNGA3 protein to stereocilia of teleost vestibular and mammalian cochlear

202 cient mechanotransduction, all the component stereocilia of the hair bundle must move essentially in

203 nnels have been localized to tips of shorter stereocilia of the mechanically sensitive hair bundle, l

204 Stereocilia of Triobp(Deltaex8/Deltaex8) mice develop no

205 The actin-filled stereocilia on each hair cell are tethered together by f

206 2 controls filament growth, stiffens exposed stereocilia, or both.

207 of this motor and its regulation within the stereocilia organelle are unknown.

208 cing, increases protein expression, improves stereocilia organization in the cochlea, and rescues coc

209 In addition, mechanotransducing shorter row stereocilia overgrow in hair cell bundles of both Cib2 m

210 mice lacking either isoform develop distinct stereocilia pathology during aging.

211 However, stereocilia patterning was grossly normal in the cochlea

212 ndle is deflected by a sensory stimulus, the stereocilia pivot as a unit, producing a shearing displa

213 was proportional to height, indicating that stereocilia pivot at their basal insertion points.

214 The forces between stereocilia produced chaotic stick-slip behavior.

215 However, stereocilia remained cohesive for deflections of up to +

216 ike filopodia, lamellipodia, microvilli, and stereocilia requires the coordinated activity of many pr

217 g the amount of free PIP2 in inner hair-cell stereocilia resulted in the following: (1) the loss of a

218 Stereocilia rootlets are angled toward the center of the

219 nd stereocilin reveal, respectively, the way stereocilia rootlets contribute to the hair bundle's mec

220 soforms can selectively traffic to different stereocilia rows.

221 lockers or disruption of tip links, leads to stereocilia shape changes and shortening.

222 n the plane of the membrane-in essence, that stereocilia shear without separation.

223 In Actg1(-/-) stereocilia similar disruptions are observed even withou

224 ous for hair cells, is in fact essential for stereocilia stability.

225 cargo, dramatically alters the slope of the stereocilia staircase in a subset of hair cells.

226 The IHC stereocilia stiffness and the gap size between the IHC s

227 nstrate that PC-1 plays an essential role in stereocilia structure and maintenance but not directly i

228 cellular processes, including maintenance of stereocilia structure, endocytosis, and autophagosome ma

229 LGN and Galphai also occupy the very tip of stereocilia that directly abut the bare zone.

230 s a relative mode of motion between adjacent stereocilia that encompasses only a fraction of a nanome

231 air cells detect sound through deflection of stereocilia that harbor mechanically-gated channels.

232 These effects occur only in stereocilia that harbor mechanotransducer channels, reco

233 ighly organized compartment near the base of stereocilia that is critical for hair cell function and

234 sma membrane-associated protein of hair cell stereocilia that is essential for hearing.

235 s expressed in the cell bodies and along the stereocilia that project from the cells' apical surface.

236 ded protein is localized along the length of stereocilia, the actin-filament-rich mechanosensory stru

237 mice, FSCN2 protein is abundant in hair-cell stereocilia, the actin-rich structures comprising the me

238 air cells detect sound through deflection of stereocilia, the microvilli-like projections that are ar

239 Stereocilia, the modified microvilli projecting from the

240 y indicates the mechanical stimulus to their stereocilia, the present results suggest that distinct l

241 escently tagged TMCs localize to the tips of stereocilia, the site of the transduction channels.

242 tructures that stimulate the outer hair cell stereocilia, the tectorial membrane and reticular lamina

243 enrichment at the bare zone confers adjacent stereocilia their tallest identity.

244 proposed to transport espin-1 to the tips of stereocilia, thereby promoting their elongation.

245 Despite the presence of many HCs with stereocilia these mice are deaf, possibly owing to HC an

246 Membrane domains within stereocilia thus define regions within hair bundles that

247 lin, and Eps8 are integral components of the stereocilia tip complex, where Eps8 is a central actin-r

248 tion of two different channels with a single stereocilia tip link.

249 Stereocilia tip links of inner ear hair cells are subjec

250 GA3 specifically to the carboxyl terminus of stereocilia tip-link protein CDH23 +68 (cadherin 23 with

251 Based upon the premise that hair cell stereocilia tip-link proteins are closely coupled with M

252 sfunction and have been shown to localize at stereocilia tips and to be essential for the elongation

253 100 copies per stereocilium, concentrated at stereocilia tips as hair cell development progressed, si

254 Only stereocilia tips had rapid turnover and no treadmilling

255 at the expression of both Eps8 and MyoXVa at stereocilia tips is reduced in whirlin-deficient mice.

256 tive hair bundle, and is concentrated toward stereocilia tips of the bundle's longest stereocilia.

257 s function to slow actin depolymerization at stereocilia tips to maintain stereocilia length.

258 rd the center of the bundle, tending to push stereocilia tips together for small deflections.

259 ng actin, which outside of a small region at stereocilia tips turns over with a very slow, months-lon

260 ocilia and increased accumulation of Eps8 at stereocilia tips, and that the exogenous expression of M

261 actively balanced between the bare zone and stereocilia tips, suggesting that early planar asymmetry

262 tion channels are located in the membrane of stereocilia tips, where the base of the tip link is atta

263 s with turnover and elongation restricted to stereocilia tips.

264 to filaments is required for localization to stereocilia tips.

265 e relies on mechanotransduction complexes at stereocilia tips.

266 sex, PIP2 localizes within stereocilia, near stereocilia tips.

267 ilopodia tips, yet is somehow able to target stereocilia tips.

268 that GFP-tagged TMC proteins localized near stereocilia tips.

269 ormly oriented with their barbed ends toward stereocilia tips.

270 hologic symmetry of the sensory organ at the stereocilia tips.

271 is properly targeted to Myo3a(-/-)Myo3b(-/-) stereocilia tips.

272 cargos that control actin polymerization at stereocilia tips.

273 and tetraspan membrane protein of hair cell stereocilia (TMHS, also known as lipoma HMGIC fusion par

274 which is consistent with the failure of OHC stereocilia to maintain stable interactions with the tec

275 sumes the stiffness of lateral links holding stereocilia together is >1000 times the pivot stiffness

276 It might be that only certain components of stereocilia turn over quickly, that rapid turnover occur

277 hair-cell stereocilia evenly, probes deflect stereocilia unevenly.

278 e abnormally tall and numerous microvilli or stereocilia, ungraded stereocilia bundles, and bundle ro

279 n, a cluster of myosin motors located at the stereocilia upper tip-link density (UTLD) keeps the tip-

280 cilia ankle-links, harmonin localizes to the stereocilia upper tip-link density and whirlin localizes

281 tes lipid membrane tented deformation in the stereocilia was developed.

282 Movement of individual stereocilia was proportional to height, indicating that

283 y linked to myosin VI at the tapered base of stereocilia, was maldistributed along the cell membrane.

284 hough tip links and staircase arrangement of stereocilia were not primarily affected by Clrn1(-/-) mu

285 in neonatal mice, in vivo and in vitro, the stereocilia were remarkably stable, incorporating newly

286 In whirlin knockout mice, the stereocilia were thickened in inner hair cells.

287 ed microvilli, by analogy to its location in stereocilia, whereas myosin XVa with the c.4351G>A or c.

288 ells tightly control the dimensions of their stereocilia, which are actin-rich protrusions with grade

289 n tightly controlled numbers of actin-filled stereocilia, which are arranged in defined rows of preci

290 Shorter rows of stereocilia, which have mechanically gated ion channels,

291 ore variable actin turnover than the tallest stereocilia, which lack channels.

292 suggest that capping protein participates in stereocilia widening by preventing newly elongating acti

293 ter shortened and disappeared; surprisingly, stereocilia width decreased concomitantly with length.

294 However, a critical subset of stereocilia with active mechanotransducer channels subse

295 Auditory sensory hair cells depend on stereocilia with precisely regulated lengths to detect s

296 inner hair cells and affects interactions of stereocilia with the tectorial membrane.

297 n-binding protein espin causes elongation of stereocilia within 12-24 hours, also suggesting rapid re

298 undle compliance or the number of functional stereocilia within a given hair bundle.

299 Individual stereocilia within each bundle contain a core of tightly

300 e located close to the tops of the hair cell stereocilia within the stereociliary bundle.