ライフサイエンスコーパス: hair cell

1 -5 hair cells; HA: 3 optic ganglion cells, 8 hair cells).

2 in the regulation of mechanotransduction by hair cells.

3 efferents innervating peripheral vestibular hair cells.

4 lls or by Atoh1-CreER(TM)-expressing type II hair cells.

5 tically in USH2 complex assembly in cochlear hair cells.

6 expanded to the entire lateral wall in outer hair cells.

7 the mechanosensitive stereociliary bundle in hair cells.

8 c80L65, shown to transduce 80-90% of sensory hair cells.

9 ies of IK,L in adult mouse vestibular type I hair cells.

10 n results in the development of ectopic root hair cells.

11 prevented the functional maturation of inner hair cells.

12 ferentiation of the implanted hESCs into new hair cells.

13 ar resident macrophages, which outnumber the hair cells.

14 oxo3 immunofluorescence in adult human outer hair cells.

15 annels at the tip of hair bundles in sensory hair cells.

16 to the mechanically sensitive stereocilia of hair cells.

17 er polar nuclear migration in root epidermal hair cells.

18 rly precise, we studied the CAPZB subunit in hair cells.

19 attached to the basolateral membrane of the hair cells.

20 as the most highly enriched ARF regulator in hair cells.

21 ells, spiral ganglion neurons and vestibular hair cells.

22 o inner ear organoids that harbor functional hair cells.

23 mbly of the mechanotransduction machinery of hair cells.

24 ials, drive somatic electromotility of outer hair cells.

25 roperties similar to those of native sensory hair cells.

26 ive electro-mechanical feedback of the outer hair cells.

27 y role in the mechanotransduction complex in hair cells.

28 ntributes to pathological changes in sensory hair cells.

29 ring thresholds, and extensive loss of outer hair cells.

30 ing role of myosin VIIa for USH2 proteins in hair cells.

31 mechanotransduction (MET) in Tomt-deficient hair cells.

32 eins play a role in USH2 complex assembly in hair cells.

33 sors have smaller apical footprints than non-hair cells.

34 to expansion of the progenitors that become hair cells.

35 cilia and kinocilia on the apical surface of hair cells.

36 ein between the frog and the mouse inner ear hair cells.

37 tently and specifically active in plant root hair cells.

38 recruitment in both low- and high-frequency hair cells.

39 porting cells phagocytose both type I and II hair cells.

40 -expressing supporting cells replace type II hair cells.

41 rge enough to account for fast adaptation in hair cells.

42 MDA-type subunits are expressed in zebrafish hair cells.

43 labeled AGs in live zebrafish mechanosensory hair cells.

44 llular architecture of cuticulosome positive hair cells.

45 nks between non-teleost electroreceptors and hair cells.

46 nd that each is expressed in developing root hair cells.

47 rentially expressed in cuticulosome positive hair cells.

48 to the cell apical region) in cochlear inner hair cells.

49 at the base of stereocilia in injectoporated hair cells, a pattern that is disrupted by deafness-asso

50 Using zebrafish neuromast hair cells, a robust model for mammalian auditory and ve

51 all changes in these values could compromise hair cells' ability to detect stimuli.

52 We have recently demonstrated that selective hair cell ablation is sufficient to attract leukocytes i

53 PCP) proteins coordinate the orientations of hair cells across the epithelial plane.

54 e first evoked action potentials (spikes) in hair-cell afferent neurons of the lateral line.

55 upporting cells can spontaneously regenerate hair cells after ablation only within the first week pos

56 ible due to large conductances that minimize hair cell and afferent time constants in the presence of

57 ble due to large conductances that minimized hair cell and afferent time constants in the presence of

58 This current depolarizes the hair cell and triggers the calcium-induced release of th

59 sin (ChR2) expressed in ear and lateral line hair cells and acquired high-speed videos of head-fixed

60 lia of outer hair cells (OHCs) but not inner hair cells and affects interactions of stereocilia with

61 POINTS: In the synaptic cleft between type I hair cells and calyceal afferents, K(+) ions accumulate

62 in the entire lateral wall of cochlear outer hair cells and had an intermediary distribution (both cy

63 anoelectrical transduction currents in outer hair cells and hence cochlear amplification is greatly r

64 onents of the mechanotransduction channel in hair cells and is essential for the transport of some of

65 vakin localizes to stereociliary rootlets in hair cells and is required for stereocilia maintenance a

66 servation accords with the function of outer hair cells and lends support to the recent hypothesis th

67 by afferent chemical neurotransmission from hair cells and modulated by efferent neurotransmitters o

68 induced peroxisome proliferation in auditory hair cells and neurons.

69 They are located in the stereocilia of hair cells and opened by the tension in specialized mole

70 Root hair cells and pollen tubes, like fungal hyphae, possess

71 For survival of cochlear hair cells and preservation of hearing, NO-mediated casc

72 hannel is expressed at the apical surface of hair cells and that it contains the Piezo2 protein.

73 t time, successful transduction of all inner hair cells and the majority of outer hair cells in an ad

74 KEY POINTS: Vestibular type I and type II hair cells and their afferent fibres send information to

75 atch-clamp recordings from turtle vestibular hair cells and their afferent neurons to show that potas

76 These include the cochlear outer hair cells and their singular feature, somatic electromo

77 ses two major types of cells, mechanosensory hair cells and underlying supporting cells, and lacks re

78 helia, the virus transduced large numbers of hair cells and virtually all the supporting cells, along

79 evel of one single plant cell type, the root hair cell, and between two model plants: Arabidopsis (Ar

80 ivery to lysosomes were immediately toxic to hair cells, and impeding lysosome delivery increased AG-

81 ary bundle, the sensory antenna of inner ear hair cells, and in the mechanoelectrical transduction pr

82 anges from nanodomain in low-frequency tuned hair cells ( approximately <2 kHz) to progressively more

83 Type I hair cells are characterized by their basolateral membra

84 Sensory hair cells are coordinately oriented within each inner e

85 the tips of sensory stereocilia of inner ear hair cells are gated by the tension of 'tip links' inter

86 Absent Foxo3, outer hair cells are lost throughout the middle and higher fre

87 ffects in vivo, we discovered that inner ear hair cells are much more vulnerable to loss of Atoh1 fun

88 lly observed shapes of hair bundles of outer hair cells are near-optimal in this regard.

89 Type I hair cells are not restored by Plp1-CreER(T2)-expressing

90 While hair bundles of inner hair cells are of linear shape, those of outer hair cell

91 Hair cells are specialized sensors located in the inner

92 support to the recent hypothesis that inner hair cells are stimulated by a net flow, in addition to

93 ABSTRACT: Type I and type II hair cells are the sensory receptors of the mammalian ve

94 Inner hair cells are transduced in an apex-to-base gradient, w

95 Cochlear hair cells are vulnerable to a variety of insults like a

96 They are also seen in wild-type hair cells around birth, appearing 2 days earlier than n

97 required for mechanotransduction in sensory hair cells as a component of the tip link.

98 acoustic overstimulation led to the loss of hair cells as well as prolonged increase in the numbers

99 hen polarized to the bare zone of individual hair cell at E13.5.

100 The ability of Anc80L65 to target outer hair cells at high rates, a requirement for restoration

101 Inner hair cells, auditory synapses and spiral ganglion neuron

102 ould increase human vulnerability to loss of hair cells because of aging or trauma.

103 nsducing shorter row stereocilia overgrow in hair cell bundles of both Cib2 mutants.

104 We find similar results in rat cochlea hair cells but extend these data to include single-chann

105 alian cochlea relies not only on the sensory hair cells, but also on the surrounding nonsensory cells

106 Here we demonstrate that loss of pejvakin in hair cells, but not in neurons, causes profound hearing

107 Destruction of hair cells causes supporting cells to generate 6 times a

108 the afferent nerve calyx surrounding type I hair cells causes unstable intercellular K(+) concentrat

109 ocilia elongation in auditory and vestibular hair cells, causing deafness and balance defects.

110 ide antibiotics are known toxins to cochlear hair cells, causing permanent hearing loss.

111 ng cells to generate 6 times as many type II hair cells compared to normal conditions.

112 In birds some hair cells contain an iron-rich organelle, the cuticulos

113 Auditory hair cells contain mechanotransduction channels that rap

114 We report here that auditory hair cells contain two molecularly distinct mechanotrans

115 where the singular organization of the outer hair cells' cortical cytoskeleton may have emerged from

116 ant spectrin, a major component of the outer hair cells' cortical cytoskeleton.

117 digms and structural modifications to reduce hair cell damage.

118 Our findings help clarify how AGs induce hair cell death and reveal properties that predict toxic

119 inst aminoglycoside antibiotic (AGA)-induced hair cell death.

120 s glutamate signaling through iGluRs induces hair-cell death independent of damage to postsynaptic te

121 Clrn1 knockout (KO) mice develop hair cell defects by postnatal day 2 (P2) and are deaf b

122 rons, causes profound hearing loss and outer hair cell degeneration in mice.

123 pontine nucleus migration defects, cochlear hair cell degeneration, and profound hearing loss.

124 With increased stimulation, hair cell depolarization increases the frequency of quan

125 function is preserved in low frequency outer hair cells, despite concomitant profound hearing loss.

126 d exhibit no mechanotransduction in auditory hair cells, despite the presence of tip links that gate

127 growth of transducing stereocilia.Inner ear hair cells detect sound through deflection of stereocili

128 Inner ear hair cells detect sound through deflection of stereocili

129 , which revealed expression in infected root hair cells, developing nodules, and in the invasion zone

130 ocilium, concentrated at stereocilia tips as hair cell development progressed, similar to the CAPZB-i

131 s of transcription factor genes critical for hair cell development, and genes essential for glutamate

132 with postnatal age, PIEZO2 may contribute to hair cell development, but it does not underlie the norm

133 equences, we identify two RBPs that regulate hair cell development.

134 ana root where they positively regulate root hair cell development.

135 nstream of Fgf signaling to not only inhibit hair cell differentiation but also to induce and maintai

136 erations in cochlear morphogenesis, auditory hair cell differentiation, and cell fate specification.

137 maturation of the mouse inner ear, cochlear hair cells display at least two types of mechanically ga

138 We therefore optimized the hair cell dissociation protocol in order to isolate matu

139 l promoter) to direct expression of Clrn1 in hair cells during development and down regulate it postn

140 plicitly-organ of Corti mechanics, and outer hair cell electro-mechanics.

141 ically significant, changes in expression of hair cell-enriched transcripts in the Gfi1(Cre) heterozy

142 e synaptic cleft, and would depolarize other hair cells enveloped by the same neuritic process increa

143 ning comes from passive mechanics within the hair cell epithelium, and that these mechanics, at least

144 This gating compliance makes hair cells especially sensitive to small stimuli.

145 ck loop that is positioned to regulate inner hair cell excitability and refine maturation of the audi

146 ir cells are of linear shape, those of outer hair cells exhibit a distinctive V-shape.

147 cell induction and demonstrate that derived hair cells exhibit electrophysiological properties simil

148 in the absence of the calyx, IK,L in type I hair cells exhibited unique biophysical activation prope

149 Finally, hair cells exposed to KA or NMDA appear to undergo apopt

150 Both type I and type ll vestibular hair cells express the alpha9 and alpha10 subunits.

151 We thus conclude that hair cells express two molecularly and functionally dist

152 le structure, leading to the hypothesis that hair cell expression of Clrn1 is essential for postnatal

153 we show that GLYCINE-RICH PROTEIN 8 promotes hair cell fate while alleviating phosphate starvation st

154 ns in a microRNA-dependent manner to inhibit hair cell fate, while also terminating growth of root ha

155 hat ELMOD1 is a GTPase-activating protein in hair cells for the small GTP-binding protein ARF6, known

156 ll fate, and is implicated in suppression of hair cell formation.

157 We found that hair cells formed first in the striolar and medial extra

158 tereocilia architecture that is critical for hair cell function.SIGNIFICANCE STATEMENT Two missense m

159 vity (FMRFamide: 4 optic ganglion cells, 4-5 hair cells; HA: 3 optic ganglion cells, 8 hair cells).

160 s that can protect or restore mechanosensory hair cells has been hampered by limited cell numbers.

161 ein-protein interaction domains of PCDH15 in hair cells has not been determined.

162 Hair cells have a unique presynaptic structure, the syna

163 est as profound changes in cell fates [short hair cells (HCs) are missing], ribbon synapse numbers, o

164 Sensory receptor hair cells (HCs) are necessary for transducing mechanica

165 dle) on the apical surface of mechanosensory hair cells (HCs) dictates the direction in which a given

166 ring and balance rely on specialized sensory hair cells (HCs) in the inner ear (IE) to convey informa

167 Yet inner hair cell (IHC) ribbons and auditory nerve responses sho

168 Ca(2+)SIGNIFICANCE STATEMENT Auditory inner hair cells (IHCs) encode sounds into nerve impulses thro

169 Inner hair cells (IHCs) in the cochlea are the mammalian phono

170 Just before the onset of hearing, the inner hair cells (IHCs) receive inhibitory efferent input from

171 t and sustained exocytosis in auditory inner hair cells (IHCs) remain largely unknown.

172 cochlear hair cells, preferentially in inner hair cells (IHCs), and was lacking from the postsynaptic

173 potential activity in immature sensory inner hair cells (IHCs), which is crucial for the refinement o

174 ribbon synapses of sensory cells, the inner hair cells (IHCs).

175 els of phosphorylated AMPKalpha increased in hair cells in a noise intensity-dependent manner.

176 tructure of efferent terminals on vestibular hair cells in alpha9, alpha10, and alpha9/10 KOs.

177 l inner hair cells and the majority of outer hair cells in an adult cochlea via virus injection into

178 h that hypothesis, perinatal transfection of hair cells in KO-TgAC1 mice with a single injection of A

179 ly efficient transduction of inner and outer hair cells in mice, a substantial improvement over conve

180 cts in Xenopus and misalignment of inner ear hair cells in mouse.

181 Outer hair cells in the cochlea have a unique motility in thei

182 tion potentials (APs) arising from the inner hair cells in the developing cochlea.

183 Vestibular hair cells in the inner ear encode head movements and me

184 toh1) governs the development of the sensory hair cells in the inner ear led to therapeutic efforts t

185 Hair cells in the mature cochlea cannot spontaneously re

186 ribbon size on synapse function, we examined hair cells in transgenic zebrafish that have enlarged ri

187 ciples to generate large numbers of bonafide hair cells in vitro.

188 ned whether glutamate excitotoxicity damages hair cells in zebrafish larvae exposed to drugs that mim

189 enerate an ATOH1-2A-eGFP cell line to detect hair cell induction and demonstrate that derived hair ce

190 In rda/rda mice, cuticular plates of utricle hair cells initially formed normally, then degenerated a

191 Stimulation of a hair cell is mediated by displacements of its mechanosen

192 o the GDP-bound form in the apical domain of hair cells is essential for stabilizing apical actin str

193 The role of mTOMT in hair cells is independent of mTOMT methyltransferase fun

194 ctional differentiation of the sensory inner hair cells is less clear.

195 One potential approach for restoring hair cells is stem cell therapy.

196 The characteristic feature of type I hair cells is the expression of a low-voltage-activated

197 ly considered as potentially deleterious for hair cells, is in fact essential for stereocilia stabili

198 Prestin in the lateral membrane of outer hair cells, is responsible for electromotility (EM) and

199 By contrast, hair cells lose contact with the basement membrane, but

200 DA), contributed to significant, progressive hair cell loss in zebrafish lateral-line organs.

201 Significant, dose-dependent hair-cell loss occurred in neurog1a morphants exposed to

202 To examine whether hair-cell loss was a secondary effect of excitotoxic dam

203 dogenous release of glutamate from the inner hair cells may increase the strength of efferent inhibit

204 Membrane trafficking in type I hair cells, measured by FM1-43 dye labeling, was altered

205 auditory stimulus to the brain, while outer hair cells mechanically modulate the stimulus through ac

206 ing protein 2 binds to the components of the hair cell mechanotransduction complex, TMC1 and TMC2, an

207 The model reproduces the main properties of hair-cell mechanotransduction using only realistic param

208 d rescue of auditory/vestibular behavior and hair cell morphology and activity.

209 Despite similarities in neuromast hair cell morphology, three classes of these cells can b

210 an low-frequency cells; high-frequency inner hair cells must have a low Ca(2+) buffer capacity to sus

211 um concentration in the cleft maintained the hair cell near potentials that promoted the influx of ca

212 Elevated potassium maintains hair cells near a potential where transduction currents

213 In sensory hair cells of auditory and vestibular organs, the ribbon

214 by molecular machinery that can vary between hair cells of different neuromasts.

215 nt near the apical junctional complex in the hair cells of mammalian ancestors and would have subsequ

216 dual manifestations of mechanosensitivity in hair cells of mouse Tmc1:Tmc2 double knockouts; 3) there

217 Hair cells of pejvakin-deficient mice develop normal roo

218 In auditory sensory hair cells of rats (Sprague Dawley) of either sex, PIP2

219 n addition, VGLUT3 is expressed in the inner hair cells of the auditory system.

220 Hair cells of the cochlea are mechanosensors for the per

221 n afferent neurons and, in the case of inner hair cells of the cochlea, vulnerability to damage from

222 time-dependent properties of IK,L in type I hair cells of the mouse semicircular canal.

223 In the auditory hair cells of young postnatal mice and rats, a reduction

224 C2 did not influence electromechanical outer hair cell (OHC) properties, as measured by distortion pr

225 at Np55 is expressed in stereocilia of outer hair cells (OHCs) but not inner hair cells and affects i

226 a is due to active forces delivered by outer hair cells (OHCs) to the cochlear partition.

227 g the cochlear spiral, contacting many outer hair cells (OHCs).

228 Achieving transduction of hair cells, or sensory neurons, throughout the cochlea h

229 ons, I exposed neurog1a morphants-fish whose hair-cell organs are devoid of afferent and efferent inn

230 ick inner ear revealed that ligand-producing hair cell precursors have smaller apical footprints than

231 CaBP2 was expressed by cochlear hair cells, preferentially in inner hair cells (IHCs), a

232 Sensory hair cells rely on otoferlin as the calcium sensor for e

233 In connexin knock-outs, inner hair cells remained stuck at a prehearing stage of devel

234 Sensory hair cells require control of physical properties of the

235 echanically sensitive hair bundle of sensory hair cells requires growth and reorganization of apical

236 f CP-AMPARs in mediating transmission at the hair cell ribbon synapse.

237 )-permeable AMPARs (CP-AMPARs) at the mature hair cell ribbon synapse.

238 However, the transfer characteristics at hair cell ribbon synapses are still poorly understood at

239 e in vivo mobility and turnover of Ribeye at hair cell ribbon synapses by monitoring fluorescence rec

240 and genes essential for glutamate release at hair cell ribbon synapses, suggesting close developmenta

241 ANCE STATEMENT Numerous studies support that hair-cell ribbon size corresponds with functional sensit

242 Together, our work indicates that hair-cell ribbon size influences the spontaneous spiking

243 Previous work has shown that hair-cell ribbon size is correlated with differences in

244 Signatures in the hair cell's behavior provide a means to determine throug

245 First, the phase of the outer hair cell's somatic force with respect to its elongation

246 ng and electrophysiology to determine that a hair cell's Tmc2b dependence is governed by neuromast to

247 S: Spontaneous activity of the sensory inner hair cells shapes maturation of the developing ascending

248 Previous work on bullfrog hair cells showed an effect of phosphoinositol-4,5-bisph

249 IFICANCE STATEMENT In the inner ear, sensory hair cells signal reception of sound.

250 the in vivo function of Pcdh15a in zebrafish hair cells.SIGNIFICANCE STATEMENT Tip links transmit for

251 (Cre) mouse is commonly used for conditional hair cell-specific gene deletion/reporter gene activatio

252 In frog hair cells, spectrin betaV was invariably detected near

253 ne results in binaural transduction of inner hair cells, spiral ganglion neurons and vestibular hair

254 s originating in the brainstem inhibit inner hair cell spontaneous activity and may further refine ma

255 Stereocilia of Fam65b-deficient murine hair cells start to develop, but mechanotransduction is

256 sensory transduction channel is expressed in hair cell stereocilia, and previous studies show that it

257 ls persistent damage to some surviving outer hair cell stereocilia.

258 Modulating the amount of free PIP2 in inner hair-cell stereocilia resulted in the following: (1) the

259 oaches in the larval zebrafish, we show that hair cell stimulation leads to robust Ca(2+) influx into

260 1 is essential for postnatal preservation of hair cell structure and hearing.

261 s important for exocytosis in high-frequency hair cells, suggesting a novel hypothesis for why these

262 GFP-tagged Tomt is enriched in the Golgi of hair cells, suggesting that Tomt might regulate the traf

263 xo3 and its transcriptional targets in outer hair cell survival after noise damage.

264 We observed higher hair cell survival rates and lower auditory brainstem re

265 emonstrate that CP-AMPARs are present at the hair cell synapse in an evolutionarily conserved manner.

266 e of noise overexposure causes damage at the hair cell synapse that later leads to neurodegeneration

267 amatergic transmission at the adult auditory hair cell synapse.

268 lar mechanism known for HHL is loss of inner hair cell synapses (synaptopathy).

269 s and the exocytosis calcium sensor at inner hair cell synapses changes along the mammalian cochlea s

270 Hair cell synapses have thus developed remarkable freque

271 s manipulation increased one type of sensory hair cell (tall HCs) at the expense of another (short HC

272 ritical gene for the development of cochlear hair cells, the receptor cells for hearing, but this has

273 ss during adaptation to prolonged stimuli by hair cells, the sensory receptors of the inner ear.

274 ximal tubule kidney cells and mechanosensory hair cells, though the mechanisms underlying cell death

275 encoding, and give further insight into how hair cells transduce signals that cover a wide dynamic r

276 relies on two types of sensory cells: inner hair cells transmit the auditory stimulus to the brain,

277 by the CCRC-M2 antibody was delayed in root hair cells (trichoblasts) compared with nonhair cells (a

278 -helix proteins are expressed in future root hair cells (trichoblasts) of the Arabidopsis thaliana ro

279 the slow Ca(2+) buffer EGTA (10 mM) in basal hair cells tuned to high frequencies ( approximately 30

280 q profiling of Arabidopsis root hair and non-hair cell types revealed extensive similarity as well as

281 emonstrate that utricular type II vestibular hair cells undergo turnover in adult mice under normal c

282 We deleted Capzb specifically in hair cells using Atoh1-Cre, which eliminated auditory an

283 an of Corti complex felt by individual outer hair cells varies along the cochlear length.

284 We determined that AGs enter hair cells via both nonendocytic and endocytic pathways.

285 the role of mcoln1 in embryonic development, hair cell viability and cellular maintenance.

286 The polarity of hair cells was established at birth along a putative lin

287 model for mammalian auditory and vestibular hair cells, we identified a urea-thiophene carboxamide,

288 Physiological properties for the outer hair cells were incorporated, such as the active force g

289 e cytoplasmic distribution in the vestibular hair cells, whereas it was detected in the entire latera

290 a MYB-driven regulatory module unique to the hair cell, which appears to control both cell fate regul

291 Functionally, hair cells with enlarged ribbons had larger global and r

292 Afferent neuron recordings revealed that hair cells with enlarged ribbons resulted in reduced spo

293 le for hair bundle integrity and labeling of hair cells with FM4-64, which was used as a proxy for me

294 Compound 90 protects mechanosensory hair cells with HC50 of 120 nM and demonstrates 100% pro

295 links the mechanical stimulation of sensory hair cells with short- and long-term signalling giving r

296 ystem is comprised of neuromasts, patches of hair cells with stereociliary bundles arranged with morp

297 ecombination that is not strictly limited to hair cells within the inner ear.

298 Moreover, hair cells within the same neuromast can break morpholog

299 n protocol in order to isolate mature type I hair cells without their calyx.

300 In this case, the absence of outer hair cells would be compatible with overexposure to unde