ライフサイエンスコーパス: hair cell

1 act as hair cell progenitors and produce new hair cells.

2 co-moves with RHD3 during tip growth of root hair cells.

3 of transducing stereocilia in mouse cochlear hair cells.

4 rting cells, which can divide and regenerate hair cells.

5 dependent pattern of root hair cells and non-hair cells.

6 ure supporting cells incapable of generating hair cells.

7 tion of neuronal genes in the GFI1-deficient hair cells.

8 y system rely on the dynamics of a system of hair cells.

9 oliferate and differentiate into replacement hair cells.

10 a the alpha9alpha10 nAChR complexes on outer hair cells.

11 upts cochlear blood flow and damages sensory hair cells.

12 ronment en route to their final targets, the hair cells.

13 iferation and differentiation as replacement hair cells.

14 ncta clustered at the synaptic pole of outer hair cells.

15 nificantly different from those of wild type hair cells.

16 ogression of support cells to differentiated hair cells.

17 ransient direct synaptic contacts with inner hair cells.

18 influx at presynaptic active zones in inner hair cells.

19 t is required only for the survival of outer hair cells.

20 in the precise orientation of mechanosensory hair cells.

21 osed of a functionally diverse population of hair cells.

22 the mechanotransduction channel of cochlear hair cells.

23 ed cochlea, together with some loss of outer hair cells.

24 ng force for sound transduction by inner ear hair cells.

25 fter supporting cells regenerate replacement hair cells.

26 sducing stereocilia in mature mouse cochlear hair cells.

27 e transducing stereocilia in mature cochlear hair cells.

28 flux, and subsequent depolarization of inner hair cells.

29 , and exosomal HSP70 interacted with TLR4 on hair cells.

30 nosensory transduction channels in inner-ear hair cells(6).

31 Here, we use cochlear hair cell ablation to isolate the effects of SNHL.

32 After hair cell ablation, YAP accumulated in supporting cell n

33 Each neuromast contains two populations of hair cells, activated by deflection in either the anteri

34 onses, vestibular-induced eye movements, and hair-cell activity as assessed with FM dye labeling and

35 ssion in cochlear hair cells than vestibular hair cells, administration of a low dose of DT caused pr

36 , differentially regulates the maturation of hair cells along the cochlea.

37 Remarkably, these hair cells also display a dynamin-dependent ultrafast en

38 Potassium sensitivity of hair cell and afferent conductances allows three modes o

39 eft ([K(+) ](c) ) contributes to setting the hair cell and afferent membrane potentials; the potassiu

40 tes potassium-sensitive conductances in both hair cell and afferent.

41 anscription factors that serve dual roles in hair cell and neuronal development (e.g. Neurod1, Atoh1

42 eta-catenin is required for specification of hair cell and supporting cell subtypes and radial patter

43 d reduced number of synapses between sensory hair cells and auditory neurons.

44 tional RBF mutants that produced ectopic non-hair cells and determined that this cell fate switch is

45 ry region, which gives rise to sound-sensing hair cells and neighboring supporting cells (SCs).

46 (pgk1) impairs Fgf-dependent development of hair cells and neurons in the otic vesicle and other neu

47 ists of a position-dependent pattern of root hair cells and non-hair cells.

48 ripotent stem cells in vitro into functional hair cells and otic-like neurons.

49 n, early specification of Myosin7-expressing hair cells and Prox1-positive supporting cells was prese

50 of spontaneous correlated activity in inner hair cells and spiral ganglion neurons, which begins at

51 ls (i.e. prosensory cells) that generate the hair cells and support cells critical for hearing functi

52 ox 2 (SOX2) is required for the formation of hair cells and supporting cells in the inner ear and is

53 or inner ear gene therapy targeting cochlear hair cells and supporting cells, and it will likely grea

54 establish sensory maps between the inner ear hair cells and the vestibular and auditory nuclei to all

55 GNs), which respond to glutamate released by hair cells and transmit auditory information into the CN

56 -pass transmembrane proteins are enriched in hair cells and underlie nonsyndromic human deafness.

57 on elicits sustained outward currents in the hair cell, and a maintained inward current in the affere

58 nsitize the channel to PIP(2) depletion from hair cells, and alter the channel's unitary conductance

59 ) modules mediate planar polarization of the hair cell apical cytoskeleton, including the kinocilium

60 Mechano-sensory hair cells are arranged in precise rows, with one row of

61 The outer hair cells are cellular actuators that are responsible f

62 To determine which type(s) of hair cells are formed postnatally, we fate-mapped neonat

63 Sensory hair cells are mechanoreceptors required for hearing and

64 In mammals, cochlear hair cells are only produced during development and thei

65 phibians, and birds which readily regenerate hair cells, are responsible in part for the mammalian ea

66 In growing root hair cells, ARK1 comets predominantly localize on the gr

67 n triggers K(+) efflux and depolarization of hair cells, as well as osmotic shrinkage of supporting c

68 high thresholds to injected currents contact hair cells at synaptic positions where neurons with high

69 mounts using confocal microscopy to quantify hair cells, auditory neurons, presynaptic ribbons, and p

70 er scale-between internal structures such as hair cells, basilar membrane (BM), and modiolus with ext

71 r findings show that most neonatally-derived hair cells become Type II, and many Type I hair cells (f

72 l mechanism for limiting the number of inner hair cells being produced.

73 tores harmonin protein expression in sensory hair cell bundles, prevents hair cell loss, improves hea

74 sensitivity and frequency selectivity of the hair cell by modulating its membrane potential.

75 plays an important role in the production of hair cells by supporting cells and provide evidence that

76 emonstrated at large appositions such as the hair cell-calyx afferent synapses present in central reg

77 or destructive, which implies that the outer hair cells can either amplify or reduce vibrations in th

78 Loss of sensory hair cells causes permanent hearing and balance deficits

79 Recent data from mammalian hair cells challenge longstanding hypotheses regarding a

80 As a result, this potassium-sensitive hair cell conductance pairs with the potassium-sensitive

81 inetic and pharmacological dissection of the hair cell conductances to understand the interdependence

82 Hair cell counts (both inner and outer) as well as frequ

83 air bundles, the mechanosensory structure of hair cells critical for hearing and balance.

84 fected cells and in vivo transduced auditory hair cells, cysteine mutagenesis experiments demonstrate

85 hair cells do not regenerate, the repair of hair cell damage is important for continued auditory fun

86 eted support cell ablation in the absence of hair cell damage.

87 Hair cell death and consequent hearing loss are common r

88 rs protection against aminoglycoside-induced hair cell death via paracrine signaling that requires ex

89 gainst aminoglycoside- and cisplatin-induced hair cell death.

90 ized ribbon synapses, and may even result in hair cell death.

91 Hearing loss correlated with cell death in hair cells, degeneration of spiral neurons and increased

92 driving force due to potassium accumulation, hair cell depolarization elicits sustained outward curre

93 Hair cell depolarization leads to calcium influx and act

94 elopmental model in which Type-I and Type-II hair cells develop in parallel rather than from an inter

95 nscription factors ATOH1, POU4F3 and GFI1 in hair cell development and regeneration, their downstream

96 opose a dual mechanism for GFI1 in promoting hair cell development, consisting of repression of neuro

97 ant FGF ligands may contribute to vestibular hair cell differentiation and supports a developmental m

98 We identify a systematic downregulation of hair cell differentiation genes, concomitant with robust

99 Given that mammalian hair cells do not regenerate, the repair of hair cell da

100 In other teleosts, OE inhibits octavolateral hair cells during locomotion.

101 ferent innervation from the CNS contacts the hair cells during this developmental window.

102 4T>G) pathogenic variant display progressive hair cell dysfunction, and that CLRN1(N48K) is trafficke

103 From a database of hair-cell-enriched translated proteins, we identify Poly

104 rgic signaling in supporting cells regulates hair cell excitability by controlling the volume of the

105 icles were sufficient to improve survival of hair cells exposed to the aminoglycoside antibiotic neom

106 ive root hair cells to instead adopt the non-hair cell fate.

107 ure striolar region of the utricle, labeling hair cells following EdU birthdating, and demonstrates t

108 rates because of acoustic pressure and outer hair cell force is critical for explaining cochlear func

109 ting from acoustic pressure and active outer hair cell force to the inner hair cells that synapse on

110 d hair cells become Type II, and many Type I hair cells (formed before P2) downregulate Sox2 and acqu

111 vide treatment targets for the protection of hair cells from chemically induced death or from other i

112 Inner hair cells from TMIE mutant mice show altered postsynapt

113 ation of peroxisomes (pexophagy) in auditory hair cells from wild-type, but not pejvakin-deficient (P

114 licylate or KCl solutions that reduced outer-hair-cell function and SFOAE amplification.

115 Despite complete loss of hair-cell function, tmc triple-mutant larvae retain norm

116 t each location.SIGNIFICANCE STATEMENT Outer hair cells generate force to amplify traveling waves wit

117 Here we demonstrate that outer hair cell-generated forces amplify traveling-wave motion

118 tic output was also heterogeneous, with some hair cells generating sustained glutamate release in res

119 o model mitotic and nonmitotic mechanisms of hair cell generation, we show that loss of LIN28B functi

120 This mechanical sense begins in hair cells grouped into neuromasts dotted along the anim

121 its effect on the mechanical response of the hair cell has not been established.

122 cular players serving efferent control of LL hair cell (HC) activity have not been identified.

123 d with gene mutations that result in sensory hair cell (HC) malfunction.

124 Each hair cell (HC) precursor of zebrafish neuromasts divides

125 Mature mammalian cochlear hair cells (HCs) do not spontaneously regenerate once lo

126 Directional sensitivity of hair cells (HCs) is conferred by the aymmetric apical ha

127 LL hair cells (HCs) share structural, functional, and molec

128 elays the differentiation of mechano-sensory hair cells (HCs).

129 ecific and early marker of Type-I vestibular hair cell identity.

130 (ARHL) is associated with the loss of inner hair cell (IHC) ribbon synapses, lower hearing sensitivi

131 nsducing stereocilia in both inner and outer hair cells (IHCs and OHCs).

132 two types of mechanotransducer cells, inner hair cells (IHCs) and outer hair cells (OHCs).

133 In the mature mammalian cochlea, inner hair cells (IHCs) are mainly innervated by afferent fibe

134 Mammalian cochlear inner hair cells (IHCs) are specialized sensory receptors able

135 Inner hair cells (IHCs) are the primary receptors for hearing.

136 Inner hair cells (IHCs) are the primary sensory receptors of t

137 We compared age-related changes in inner hair cells (IHCs) between four mouse strains with differ

138 current is prominent, and in mammalian inner hair cells (IHCs) displays unusual properties.

139 The GFP reporter showed that inner hair cells (IHCs) were transfected throughout the cochle

140 mice, outer hair cells (OHCs), but not inner hair cells (IHCs), began to lose their third row of ster

141 al ganglion neurons (SGNs) on cochlear inner hair cells (IHCs), resulting in loss of synapses, a proc

142 r to the primary sensory receptor, the inner hair cells (IHCs), the mature functional characteristics

143 day 7 (P7), before the primary sensory inner hair cells (IHCs), which become competent at about the o

144 ouse), MYO7A is severely diminished in inner hair cells (IHCs), while expression in outer hair cells

145 Hair cells in both the auditory and vestibular systems r

146 utamatergic transmission from cochlear inner hair cells in mice lacking the vesicular glutamate trans

147 r (MET) currents were recorded from cochlear hair cells in mice with mutations of transmembrane chann

148 While hair cells in the cochlea are established targets of cis

149 en due to the absence or the degeneration of hair cells in the cochlea.

150 ic drugs, infections, and aging kill sensory hair cells in the ear, causing irreversible hearing loss

151 The mechanoreceptive sensory hair cells in the inner ear are selectively vulnerable t

152 sted this effect on in vitro preparations of hair cells in the sacculi of American bullfrogs of both

153 Dopamine and L-mimosine protected the hair cells in the zebrafish otic vesicle from cisplatin-

154 Growing hair cells in vitro would provide a route to overcome th

155 a type of sensory receptor cells (the outer hair cells) in response to the acoustic vibrations.

156 and post-synaptic markers on cochlear inner hair cells, in guinea pigs surviving from 1 day to 6 mon

157 maturation, growth and innervation of inner hair cells; in contrast, it is required only for the sur

158 Type I hair cells influence discharge rates in their calyx affe

159 -GEF-Rac axis mediates both tissue-level and hair cell-intrinsic PCP signaling.

160 r planar cell polarity (PCP) signaling and a hair cell-intrinsic, microtubule-mediated machinery.

161 hair cells (IHCs), while expression in outer hair cells is affected tonotopically.

162 t the development of low- and high-frequency hair cells is differentially regulated during developmen

163 t the development of low- and high-frequency hair cells is differentially regulated during pre-hearin

164 The maintenance of hair cells is further challenged by damage from a variet

165 The hair bundle of cochlear hair cells is the site of auditory mechanoelectrical tra

166 ies despite normal prestin function in outer hair cells isolated from naked mole-rats.

167 In mammals, hair cells lack regenerative capacity, and their death l

168 Loss of sensory hair cells leads to deafness and balance deficiencies.

169 and postnatal supporting cells into induced hair cell-like cells (iHCs).

170 iHCs exhibit hair cell-like morphology, transcriptomic and epigenetic

171 posed to a loud noise event that resulted in hair cell loss and reduced hearing capability had a supr

172 responsible, in part, for the permanence of hair cell loss in mammals.

173 e-related cochlear synaptic degeneration and hair cell loss in mice with enhanced alpha9alpha10 choli

174 ture cochlea, prior to the onset of hearing, hair cell loss stimulates neighboring supporting cells t

175 atform to identify causes and treatments for hair cell loss, and may help identify future gene therap

176 ssion in sensory hair cell bundles, prevents hair cell loss, improves hearing sensitivity, and amelio

177 bstantial nerve fiber demyelination and mild hair cell loss.

178 elia in vivo drives cell cycle reentry after hair cell loss.

179 rd manifestation of hair bundle disarray and hair cell loss.

180 Sensory hair cell losses underlie the vast majority of permanent

181 ototoxic drugs, infections, and aging cause hair cell losses.

182 ay also use prestin at high frequencies, but hair cells <1 kHz show electrical resonance.

183 ransfected throughout the cochlea, and outer hair cells mainly in the apex.

184 cells, which produced progeny that expressed hair cell markers, but proliferative responses declined

185 enhancers of Pbx1, Fgf8, Dusp6, Vangl2, the hair-cell master regulator Atoh1 and a cascade of Atoh1'

186 The finite number of hair cells means that the cochlea itself can be thought

187 form the tip-links, whose tension gates the hair cell mechanoelectrical transduction channels.

188 at the C-subtypes both bind and permeate the hair cell mechanotransducer channel, with the stronger t

189 sed to function as a motor that tensions the hair cell mechanotransduction (MET) complex, but conclus

190 MC1 has been shown to constitute the pore of hair cell mechanotransduction channels, but little is kn

191 ifest in many forms, from dysfunction of the hair cell mechanotransduction complex to loss of special

192 f its integrity for long-term maintenance of hair cell mechanotransduction, are not known.

193 Synaptic puncta move all over the hair cell membrane during recovery, translocating far fr

194 hts MYO7A's essential role in tensioning the hair cell MET complex.

195 sults suggest that fluid motion due to outer hair cell motility can help maintain longitudinal homeos

196 f potassium ion concentration; second, outer hair cell motility causes organ of Corti deformations th

197 dressed for a comprehensive understanding of hair cell MT at molecular and atomic levels.

198 nt advances, we propose a unifying theory of hair cell MT that may reconcile most of the functional d

199 ectrical transduction (MET) in the inner-ear hair cells of larval zebrafish.

200 brane Channel-Like (Tmc) 1 or 2 into sensory hair cells of mice with hearing and balance deficits due

201 HA immunopuncta also occurred in inner hair cells of pre-hearing (P7) but not in adult mice.

202 Hearing loss caused by the death of sensory hair cells of the inner ear is an unfortunate side effec

203 duction (MT) machinery in highly specialized hair cells of the inner ear.

204 Since inner ear outer hair cell (OHC) degeneration is a common trait of age-re

205 Outer hair cell (OHC) nonlinear capacitance (NLC) represents v

206 The amplifier relies on outer hair cell (OHC)-generated forces driven in part by the e

207 Cochlear outer hair cells (OHCs) are among the fastest known biological

208 Outer hair cells (OHCs) are electromotile sensory receptors th

209 Outer hair cells (OHCs) enhance the sensitivity and the freque

210 The effectiveness of outer hair cells (OHCs) in amplifying the motion of the organ

211 Outer hair cells (OHCs) in the mammalian ear exhibit electromo

212 In Baiap2l2 deficient mice, outer hair cells (OHCs), but not inner hair cells (IHCs), bega

213 The function of outer hair cells (OHCs), the mechanical actuators of the cochl

214 pends upon specialized hair cells, the outer hair cells (OHCs), which possess both sensory and motile

215 cer cells, inner hair cells (IHCs) and outer hair cells (OHCs).

216 ectromechanical properties of cochlear outer hair cells (OHCs).

217 s, including those associated with the outer hair cells (OHCs).

218 holine receptors (nAChRs), which assemble in hair cells only coincident with cholinergic innervation

219 The remaining hair cells only signaled motion in one direction and wer

220 d to tonotopic variations in the constituent hair cells or cytoarchitecture of the organ of Corti.

221 he planar cell polarity (PCP) pathway aligns hair cell orientation along the plane of the sensory epi

222 e copy signal acted with high selectivity on hair cells polarized to be activated by posterior deflec

223 Newly-generated hair cells presented in three cochlear turns and were ab

224 EUROD1 regulon and are normally expressed in hair cells prior to GFI1 onset.

225 We show that burst firing of mouse inner hair cells prior to hearing onset requires P2RY1 autorec

226 s crucial for identifying factors to trigger hair cell production in mammals.

227 lates neighboring supporting cells to act as hair cell progenitors and produce new hair cells.

228 that Plp1+ supporting cells took on type II hair cell properties based on molecular markers, basolat

229 Auditory hair cells receive olivocochlear efferent innervation, w

230 tion mode of the hair bundle will affect the hair cell receptor current and stimulus coding.

231 Lowering R(in) will reduce the hair cells receptor potential and presumably moderate th

232 Solution effects on inner hair cells reduced auditory nerve compound action potent

233 st that the Dnmt inhibitor 5-aza may promote hair cell regeneration in a chemically-deafened mouse mo

234 scRNA-Seq analyses of fgf3 mutants, in which hair cell regeneration is increased, demonstrates that F

235 Mature mammalian auditory hair cells require transmembrane channel-like 1 (TMC1) f

236 Mechanoelectrical transduction at auditory hair cells requires highly specialized stereociliary bun

237 The bundle of stereocilia on inner ear hair cells responds to subnanometer deflections produced

238 potentials; the potassium efflux from type I hair cells results from the interdependent gating of thr

239 for synaptic vesicle exocytosis at auditory hair cell ribbon synapses.

240 auditory-nerve terminals extend towards the hair cell's apical end to re-establish contact with them

241 e is ascribed to the saturation of the outer hair cell's mechano-transduction.

242 we found, may be mediated by a block of the hair cell's mechanoelectrical transducer (MET) channel,

243 Half the hair cells signaled cupula motion in both directions fro

244 ature in Ca(V) 1.3(-/-) mice was the reduced hair cell size irrespective of their cochlear location.

245 ith one row of inner and three rows of outer hair cells spanning the length of the spiral-shaped sens

246 architecture, and perturbs transcription of hair cells specific genes during zebrafish development.

247 toxin (DT) receptor was expressed behind the hair-cell specific Pou4f3 promoter.

248 Hair cell-specific expression of the known HSP70 recepto

249 Transgenic, hair cell-specific expression of Tmc2b-mEGFP rescues the

250 al-associated genes as well as activation of hair cell-specific genes required for normal functional

251 d mouse cochleas, we demonstrated that inner hair cell stereocilia developed in specific stages, wher

252 lectrical transduction process occurs in the hair-cell stereocilia of the inner ear, which experience

253 rical transduction (MET) channels at tips of hair-cell stereocilia.

254 In developing and mature sensory hair cells, stereocilia are connected to each other by v

255 eriments reveal that, in developing cristae, hair cells stratify into an upper, Tmc2a-dependent layer

256 Here, we discuss how several key hair cell structures can be damaged, and what is known a

257 The utricle consists of two hair cell subtypes with distinct morphological, electrop

258 membrane currents in low-frequency (apical) hair cells, such as I(K,n) (carried by KCNQ4 channels),

259 y expressed genes in auditory and vestibular hair cells suggests that GFI1 serves different roles in

260 veal Pappaa as an extracellular regulator of hair cell survival and essential mitochondrial function.

261 the inner ear that can mediate nonautonomous hair cell survival.

262 ers the pleiotropic role of otoferlin in the hair cell synaptic vesicle cycle, notably in triggering

263 pses between auditory nerve fibers and their hair cell targets without destroying the hair cells them

264 Due to higher Pou4f3 expression in cochlear hair cells than vestibular hair cells, administration of

265 ey extend a peripheral axon beyond the inner hair cells that subsequently makes a distinct 90 degree

266 nd active outer hair cell force to the inner hair cells that synapse on afferent nerves.

267 Hair cells, the mechanosensory receptors of the inner ea

268 e mammalian cochlea depends upon specialized hair cells, the outer hair cells (OHCs), which possess b

269 Hair cells, the sensory receptors of the inner ear, resp

270 the forced overexpression may be harmful to hair cells themselves during cochlear overstimulation.

271 eir hair cell targets without destroying the hair cells themselves.

272 uditory/vestibular end organs and subsets of hair cells therein rely on distinct combinations of Tmc1

273 the medial olivary complex inhibit cochlear hair cells through the activation of alpha9alpha10-conta

274 Afferent synapses were lost from inner hair cells throughout the aged cochlea, together with so

275 s disrupted while still allowing a subset of hair cells to detect stimuli originating in the external

276 IO23 (APUM23), which caused prospective root hair cells to instead adopt the non-hair cell fate.

277 t engaged the Toll-like receptor 4 (TLR4) on hair cells to protect them from death.

278 t the tips of the tall stereocilia in mature hair cells, together with PCDH15 isoforms CD1 and CD2; L

279 ts for future drug design and mechanisms for hair cell toxicity.

280 cre);RiboTag mice to evaluate changes to the hair cell translatome in the absence of GFI1.

281 stronger deflections.SIGNIFICANCE STATEMENT Hair cells transmit information about mechanical stimuli

282 Here, I review the various hair cell-tuning mechanisms used among vertebrates.

283 The receptive field maps of hair cells undergoing efferent actuation demonstrated an

284 ng in mammals uses somatic motility of outer hair cells, underpinned by the membrane protein prestin,

285 nd transient burst of glutamate release from hair cells unresponsive to the initial stimulus.

286 fluorescent protein and find that different hair cells vary in their mechanical sensitivity and the

287 te into progenitor-like cells and to produce hair cells via mitotic and nonmitotic mechanisms.

288 The maturation of high-frequency (basal) hair cells was also affected in Ca(V) 1.3(-/-) mice, but

289 correlate with synaptic position on sensory hair cells, we combined patch clamping with fiber labeli

290 Cochlear hair cells were conditionally and selectively ablated in

291 Immunolabeled hair cells were used to visualize the spiraling BM in th

292 rations, including those of individual outer hair cells, were measured using optical coherence tomogr

293 In contrast, zebrafish lateral line hair cells, which detect water motion, require Tmc2a and

294 ngly, Tmc1(KO/KO);Tmc2(KO/KO) or Tmie(KO/KO) hair cells, which lack transduction, have significantly

295 sorineural components of the cochlea include hair cells, which respond mechanically to sound waves, a

296 We studied both inner and outer hair cells, which send nervous signals to the brain and

297 A population of hair cells with these different sensitivities, operating

298 dles that project from the apical surface of hair cells within the cochlea.

299 Mechano-sensory hair cells within the inner ear cochlea are essential fo

300 orm mechanotransduction channels in cochlear hair cells without TMIE.