ライフサイエンスコーパス: inner ear

1 ene deletion/reporter gene activation in the inner ear.

2 ransducer (MET) channel in hair cells of the inner ear.

3 endolymph in the membranous labyrinth in the inner ear.

4 ghts that mediate mechanotransduction in the inner ear.

5 (SGNs) are specialzed bipolar neurons in the inner ear.

6 with limited cell type diversification, the inner ear.

7 is the mechano-transduction apparatus of the inner ear.

8 (N48K) mutation in sensory hair cells of the inner ear.

9 imination of XIRP2 protein expression in the inner ear.

10 ium secretion by the stria vascularis in the inner ear.

11 terize hair-cell mechano-transduction in the inner ear.

12 of the small number of cells present in the inner ear.

13 oidance of further damage to the irreparable inner ear.

14 lea is a highly specialized organ within the inner ear.

15 y and frequency selectivity of the mammalian inner ear.

16 ole for GNAQ in melanocyte-like cells of the inner ear.

17 at it is ubiquitously expressed in the mouse inner ear.

18 re in hair cells and auditory neurons of the inner ear.

19 translate PCP cues for BB positioning in the inner ear.

20 ntrol of a nerve growth factor (BDNF) in the inner ear.

21 the organ of Corti within the cochlea of the inner ear.

22 listener using an electrode implanted in his inner ear.

23 e of hair-bundle structure in the developing inner ear.

24 rogenitors for most cell types of the mature inner ear.

25 ring to the deaf by directly stimulating the inner ear.

26 s important to hair cell function within the inner ear.

27 ir cells are the mechanosensory cells of the inner ear.

28 acting as responders to inflammation in the inner ear.

29 eocilia of inner and outer hair cells of the inner ear.

30 inhibit sensory hair cells of the vertebrate inner ear.

31 e application on the delivery of GENT to the inner ear.

32 ty and frequency resolution to the mammalian inner ear.

33 h enlarged endolymphatic compartments of the inner ear.

34 ot strictly limited to hair cells within the inner ear.

35 by hair cells, the sensory receptors of the inner ear.

36 nd saccule, the main hearing endorgan of the inner ear.

37 he otic vesicle, the embryonic anlage of the inner ear.

38 nd research on therapies for diseases of the inner ear.

39 rce of cells for cell-based therapies of the inner ear.

40 rgy to enhance mechanical oscillation in the inner ear.

41 al for overall development of HCs within the inner ear.

42 lysaccharide into the scala tympani of mouse inner ears.

43 her syndrome corrects gene expression in the inner ear, a therapeutically relevant target tissue.

44 observed, with additional eye, skeletal and inner ear abnormalities characterized in the bpck mouse.

45 The effects of inner ear abnormality on audibility have been explored s

46 then propose a diagnostic method to identify inner ear alterations in stranded individuals.

47 a/leukemia), as well as for neoplasms of the inner ear and cranium, and b) there is consistency and v

48 (ES) is a cystic organ that is a part of the inner ear and is connected to the cochlea and vestibule.

49 in outer hair cells (OHCs) of the mammalian inner ear and is required for cochlear amplification, a

50 r cell regeneration in vivo in the zebrafish inner ear and lateral line.

51 ell proliferation of supporting cells in the inner ear and lateral line.

52 D1b, tightly localizes to ribbon synapses in inner ear and lateral-line hair cells.

53 pamine in mechanosensory organs, such as the inner ear and lateral-line organ, is not clearly underst

54 ivo results in stunted sensory organs of the inner ear and loss of hair cells.

55 nnels are expressed in the kidney and in the inner ear and require the accessory subunit barttin for

56 l region disrupted FL-whirlin isoform in the inner ear and retina but not C-whirlin in the inner ear,

57 Functional class analysis showed that inner ear and schizophrenia-related genes were enriched

58 fferent versus efferent circuits between the inner ear and the brain.

59 sorganized stereociliary bundles in the bpck inner ear and the convergent extension defects in zebraf

60 characteristics of DA fibers innervating the inner ear and the hindbrain auditory efferent nucleus in

61 mation of the distinct sensory organs of the inner ear and the non-sensory domains that separate them

62 cells (RGC), spiral and vestibular ganglia, inner ear and vestibular hair cell neurons in the vestib

63 w that miR-224 and Ptx3 are expressed in the inner ear and we demonstrate that miR-224 targets Ptx3.

64 any genes are misregulated in the diminuendo inner ear and we report here further misregulated genes.

65 novel role for Rsph9 in the kinocilia of the inner ear and/or lateral line neuromasts.

66 e upper urinary system, cardiac venous pole, inner ear, and axial skeleton; homozygous null mutant an

67 the cells of the lens, retina, heart muscle, inner ear, and bone are dependent on XylT2 for proteogly

68 rent ribbon synapses in sensory cells of the inner ear, and for normal hearing.

69 nner ear and retina but not C-whirlin in the inner ear, and led to retinal degeneration as well as mo

70 hanosensory hair cells (HCs) residing in the inner ear are critical for hearing and balance.

71 Hair cells of the inner ear are essential for hearing and balance.

72 use the fragile membranous structures in the inner ear are hard to visualize undistorted and in full.

73 The hair cells of the mammalian inner ear are the mechanosensors for the detection of so

74 de their hearing sensitivity range and their inner ears are partly undeveloped, which accounts for th

75 In vertebrates, the inner ear arises from the otic placode, a thickened swat

76 R-135b in vestibular hair cells of the mouse inner ear as well as a possible protector against photor

77 ion we conditionally deleted the gene in the inner ear at various embryonic and postnatal ages.

78 In the inner ear, at least two systems regulate the planar pola

79 tion in mice we found that, in the postnatal inner ear, Bbnf and Ntf3 are required for the formation

80 le mutants, they extend more dorsally in the inner ear beyond normal vestibular target areas.

81 In the mammalian inner ear, bicellular and tricellular tight junctions (t

82 basic and translational investigations into inner ear biology and regeneration.

83 an involve the musculoskeletal system, skin, inner ear, brain, and spinal cord, with characteristic s

84 ctions to sensory epithelia in the embryonic inner ear, but their postnatal functions remain poorly u

85 vaccination, the patches were applied to the inner ear by hand without an applicator.

86 construction of a complex sensory organ, the inner ear, by imaging zebrafish embryos in vivo over an

87 Because the human inner ear cannot be visualized during life, histopatholo

88 the developmental processes that form unique inner ear cell types.

89 hearing loss occur at the cellular level and inner ear cells are very sensitive to autolysis.

90 Here in vivo clonal analysis of mouse inner ear cells during development demonstrates clonal r

91 lators of differentiation and development of inner ear cells.

92 that KARS variants affect aminoacylation in inner-ear cells by interfering with binding activity to

93 ut does possess the highly derived brain and inner ear characteristic of the latest Cretaceous specie

94 hano-sensory hair cells (HCs), housed in the inner ear cochlea, are critical for the perception of so

95 In the inner ear, cochlear and vestibular sensory epithelia uti

96 Throughout postnatal maturation of the mouse inner ear, cochlear hair cells display at least two type

97 The aims of this study were to manipulate inner ear connexin expression in vivo using BAAV vectors

98 The inner ear consists of two otocyst-derived, structurally

99 The inner ear contains sensory epithelia that detect head mo

100 Histopathologic study of the human inner ear continues to emphasize the need for non- or mi

101 e for most of the syndromic defects, exhibit inner ear defects and hyperactivity.

102 neural crest stream adjacent to r5, and have inner ear defects.

103 the mechanisms underlying inherited forms of inner ear deficits has considerably improved during the

104 's disease (MD) is a chronic disorder of the inner ear defined by sensorineural hearing loss, tinnitu

105 ions of an extracellular tissue found in the inner ear demonstrating a mechanism of frequency separat

106 The vestibular system of the inner ear detects head position using three orthogonally

107 h could be used to investigate mechanisms of inner ear development and disease as well as regenerativ

108 entify new gene interactions responsible for inner ear development and for the segregation of the oti

109 MEKK4) expression is highly regulated during inner ear development and is critical to normal cytoarch

110 se, Adcy1 expression was observed throughout inner ear development and maturation.

111 system should facilitate the study of human inner ear development and research on therapies for dise

112 rentiation is crucial not only to understand inner ear development but also to improve cell replaceme

113 Zebrafish inner ear development is characterized by the crystalliz

114 plex interplay between alternative splicing, inner ear development, and auditory function.

115 milies involved in barrier function, eye and inner ear development, insect coagulation and innate imm

116 NA-Seq study of gene expression during mouse inner ear development.

117 ant factor for initiation and elaboration of inner ear development.

118 dal ectoderm, two critical precursors during inner ear development.

119 nct progenitor populations from the neonatal inner ear differentiate to cell types associated with th

120 Autoimmune inner ear disease (AIED) is a rare disease that results

121 ed with drugs commonly used for treatment of inner ear diseases, significantly improving the drugs' s

122 table for translation to humans with genetic inner ear disorders.

123 h as the otic placode that gives rise to the inner ear do not exist.

124 for the first time, sustained and controlled inner ear drug delivery can be successfully regulated en

125 We have recently developed a novel inner ear drug delivery system using chitosan glyceropho

126 Here we present a new approach to inner ear drug delivery with induced advection aiding di

127 sizing enzyme and is highly expressed in the inner ear during embryogenesis.

128 findings highlight a biological link between inner ear dysfunction and behavioral disorders and how s

129 pproaches in mice, the authors show that (1) inner ear dysfunction due to either Tbx1 or Slc12a2 muta

130 Furthermore, we show that (1) inner ear dysfunction due to the tissue-specific loss of

131 gs reveal that a sensory impairment, such as inner ear dysfunction, can induce specific molecular cha

132 ther hyperactivity or anxiety co-occurs with inner ear dysfunction.

133 ure hyperactivity in individuals with severe inner ear dysfunction.

134 hether hyperactivity or anxiety coexist with inner ear dysfunction.

135 d during optokinetic reflex compensation for inner ear dysfunction.

136 In the inner ear, EF-hand calcium buffers may play a significan

137 Vestibular hair cells in the inner ear encode head movements and mediate the sense of

138 bicellular and tricellular junctions in the inner ear epithelia of tricellulin-deficient mice.

139 howed that both genes are expressed in mouse inner ear, especially in hair cells, further suggesting

140 Maintenance of the composition of inner ear fluid and regulation of electrolytes and acid-

141 aintenance of the unique ionic milieu of the inner ear fluid.

142 ics based on the morphology, dynamics of the inner ear fluids, and membranous labyrinth deformability

143 cally expressed during early stages of chick inner ear formation.

144 only in the juvenile lateral line and in the inner ear from >2 months after hatching.

145 also regenerate hair cells, we investigated inner ears from dogfish sharks, zebrafish, bullfrogs, Xe

146 Inner ears from two mass strandings of long-finned pilot

147 adequately activate genes crucial for normal inner ear function and acid-base regulation in the kidne

148 The data represent unprecedented recovery of inner ear function and suggest that biological therapies

149 ride cotransporter and is also necessary for inner ear function, causes hyperactivity; (2) vestibular

150 gene replacement as a strategy for restoring inner ear functions in a mouse model of Usher syndrome t

151 The use of viral vectors for inner ear gene therapy is receiving increased attention

152 To understand how the inner ear-generated sound, i.e., otoacoustic emission, e

153 non-human cochlear specimens regulating the inner ear glutamate concentration.

154 nly used anticancer drugs, is known to cause inner ear hair cell damage and hearing loss.

155 ction is required for the earliest stages of inner ear hair cell development, which begins during the

156 The precise assembly of inner ear hair cell stereocilia into rows of increasing

157 ith a pseudostratified epithelium containing inner ear hair cells and supporting cells after 16-20 d.

158 annels at the tips of sensory stereocilia of inner ear hair cells are gated by the tension of 'tip li

159 ates its effects in vivo, we discovered that inner ear hair cells are much more vulnerable to loss of

160 miting the growth of transducing stereocilia.Inner ear hair cells detect sound through deflection of

161 Inner ear hair cells detect sound through deflection of

162 ilage defects in Xenopus and misalignment of inner ear hair cells in mouse.

163 Deafness caused by the terminal loss of inner ear hair cells is one of the most common sensory d

164 myosin 15 is a molecular motor expressed in inner ear hair cells that transports protein cargos with

165 Inner ear hair cells transduce sound waves and accelerat

166 stereociliary bundle, the sensory antenna of inner ear hair cells, and in the mechanoelectrical trans

167 wn cells of the embryonic node, kinocilia of inner ear hair cells, and several cell lines.

168 y for kinocilium positioning in monociliated inner ear hair cells.

169 this protein between the frog and the mouse inner ear hair cells.

170 The hair bundle--the sensory organelle of inner-ear hair cells of vertebrates--exemplifies the abi

171 Tip link filaments convey force and gate inner-ear hair-cell transduction channels to mediate per

172 The human inner ear has an intricate spiral shape often compared t

173 Hair bundles of the inner ear have a specialized structure and protein compo

174 oaches to introduce viral particles into the inner ear have been described, presumed physiological ba

175 and balance organs of the neonatal mammalian inner ear have the capacity to generate new hair cells a

176 a model to study HC development and to drive inner ear HC regeneration.

177 ical stimulation of vestibular organs in the inner ear helps to dissociate vestibular impairments tha

178 o neurodegenerative disease, yet its role in inner ear homeostasis and hearing loss is essentially un

179 necessary for sensory hair cell survival and inner ear homeostasis.

180 ear (TME) that transmits sound waves to the inner ear; however, numerous species lack some or all TM

181 The mammalian inner ear (IE) subserves auditory and vestibular sensati

182 specialized sensory hair cells (HCs) in the inner ear (IE) to convey information about sound, accele

183 specialized sensory hair cells (HCs) in the inner ear (IE) to convey information about sound, accele

184 ing distribution of compounds throughout the inner ear in the murine cochlea.

185 vity in hair cells from the lateral line and inner ear in vivo and using near-physiological in vitro

186 nase and Cas9:sgRNA complexes into the mouse inner ear in vivo, achieving 90% Cre-mediated recombinat

187 We report that inflammatory stimuli in the inner ear induce activation of the innate immune system

188 The system faithfully recapitulates mouse inner ear induction followed by self-guided development

189 cs for reducing inflammation associated with inner ear injury.

190 ggest that KDM4B play a critical role during inner ear invagination via modulating histone methylatio

191 The ES is thought to be involved in inner ear ion homeostasis and fluid volume regulation fo

192 Mechanoelectrical transduction in the inner ear is a biophysical process underlying the senses

193 The inner ear is a complex vertebrate sense organ, yet it ar

194 e semicircular canal ducts in the vertebrate inner ear is a dramatic example of epithelial remodellin

195 important for mechanotransduction within the inner ear, is catalyzed by adenylate cyclases (AC).

196 pattern the sensorineural components of the inner ear, its role in middle ear development has been l

197 how pitch information is extracted from the inner ear itself.

198 t time and that lack of HGF signaling in the inner ear leads to profound hearing loss in the mouse.

199 It is known that damage to the inner ear leads to recruitment of inflammatory cells (ma

200 development of the sensory hair cells in the inner ear led to therapeutic efforts to restore these ce

201 ete phenotypic conversion of stem cells into inner-ear-like cells.

202 rol over ionic composition and volume of the inner ear luminal fluid endolymph is essential for norma

203 ects with valve dysplasia, and deafness with inner ear malformations.

204 molecules and reduced expression of several inner ear markers, including Dlx3.

205 mechanosensor, implicating them directly in inner ear mechanosensation.

206 So far, attempts to derive inner ear mechanosensitive hair cells and sensory neuron

207 inkers and suggests how they may function in inner-ear mechanotransduction, with implications for oth

208 darker-skinned individuals tend to have more inner ear melanin, and cochlear melanocytes are importan

209 g evidence of an essential role for MEKK4 in inner ear morphogenesis and identifies the requirement o

210 genized Xenopus tropicalis has identified an inner ear mutant named eclipse (ecl).

211 Using immunofluorescence on rat inner ear neuroepithelia, DCDC2a was found to localize t

212 Here we tested SOX2's requirement during inner ear neuronal specification using a conditional del

213 facilitate fast synaptic transmission in the inner ear of frogs (Rana catesbeiana and Rana pipiens).

214 BE3 RNPs into both zebrafish embryos and the inner ear of live mice to achieve specific, DNA-free bas

215 s cDNA by the adenoassociated virus 8 to the inner ear of newborn mutant mice reestablishes the expre

216 Therefore, the pathological changes in the inner ear of the mice with SNHL supported the phenotypic

217 haracterize CA distribution in the brain and inner ear of the plainfin midshipman fish (Porichthys no

218 d their receptors have been localized to the inner ear of the rat and guinea pig mammalian models.

219 iency, we delivered wild-type Ush1c into the inner ear of Ush1c c.216G>A mice using a synthetic adeno

220 neration is induced by laser ablation in the inner ear or by neomycin treatment in the lateral line,

221 ferentiating human pluripotent stem cells to inner ear organoids that harbor functional hair cells.

222 Over 2 months, the vesicles develop into inner ear organoids with sensory epithelia that are inne

223 supporting cells and sensory-like neurons as inner ear organoids.

224 could have facilitated the emergence of new inner ear organs and their functional diversification in

225 y role played by the two low-frequency-tuned inner-ear organs in anuran amphibians - the amphibian pa

226 acoustic Ganglion (SAG), which innervate the inner ear, originate as neuroblasts in the floor of the

227 Their counterparts in the mammalian inner ear, otoconia, have a primarily vestibular functio

228 Among inner ear pathologies, inflammation may lead to structur

229 th these findings, GENT concentration in the inner ear perilymph was significantly decreased after ch

230 nt effect that genetic background has on the inner ear phenotype of Atp6v1b1 mutant mice provides ins

231 The abnormal inner ear phenotype of MRL- Atp6v1b1vtx/vtx mice was los

232 recombinase in the Gfi1(Cre) mouse neonatal inner ear, primarily in inner ear resident macrophages,

233 d resolution to obtain information about the inner ear prior to performing surgery.

234 Specification of inner ear progenitors is initiated by FGF signalling.

235 formation in the brain, we investigated the inner ear projection, known for its well-defined and ear

236 The inner ear receives two types of efferent feedback from t

237 ss highly disparate macromorphologies of the inner ear relative to each other and to mammals, thereby

238 Sensory perception in the inner ear relies on the hair bundle, the highly polarize

239 faces with high anatomic detail, such as the inner ear, remain a challenge.

240 acrophages and the sensory structures of the inner ear remains unclear.

241 sease as well as regenerative mechanisms for inner ear repair.

242 (Cre) mouse neonatal inner ear, primarily in inner ear resident macrophages, which outnumber the hair

243 ls (HCs) are the mechanoreceptors within the inner ear responsible for our sense of hearing.

244 his prediction, analysis of developing chick inner ear revealed that ligand-producing hair cell precu

245 n of ~P23 mouse vestibular hair bundles, the inner ear's sensory organelle.

246 Because dimension control of the inner ear's stereocilia is particularly precise, we stud

247 Inner ear sensory epithelia contain mechanosensitive hai

248 ver, tricellulin becomes mislocalized in the inner ear sensory epithelia of ILDR1 null mice after the

249 ion, and stereociliary bundle orientation in inner ear sensory hair cells - appear to be mechanistica

250 xpressed in central auditory pathways and in inner ear sensory hair cells and skeletal and smooth mus

251 cells are coordinately oriented within each inner ear sensory organ to exhibit a particular form of

252 is asymmetrically localized in cells of the inner ear sensory organs, characteristic of components o

253 zation of ES ion transport cells relative to inner ear sensory organs, dysplasia of the endolymph flu

254 he loss of mAnkrd6 causes PCP defects in the inner ear sensory organs.

255 lesce to form the neurons that innervate all inner ear sensory regions.

256 reproducible and scalable means to generate inner ear sensory tissue in vitro.

257 protocol describes a culture system in which inner-ear sensory tissue is produced from mouse embryoni

258 d cytotoxicity.SIGNIFICANCE STATEMENT In the inner ear, sensory hair cells signal reception of sound.

259 The mammalian inner ear separates sounds by their frequency content, a

260 Hair cells from the inner ear showed a developmental change in current profi

261 eins in cell signaling in general and in the inner ear specifically, and open the door to exploration

262 rmation of kidney and intestinal microvilli, inner ear stereocilia, immune synapses, endocytic patche

263 ic sac is required for acquisition of normal inner ear structure and function.

264 Certain viral infections can directly damage inner ear structures, others can induce inflammatory res

265 derived neurotrophins play critical roles in inner ear synapse density and synaptic regeneration afte

266 acellular proteins, as egg coat proteins and inner ear tectorins.

267 the specialized mechanosensory cells of the inner ear that capture auditory and balance sensory inpu

268 cells are specialized sensors located in the inner ear that enable the transduction of sound, motion,

269 ROS) within mechanosensory hair cells of the inner ear that have been implicated in hearing and balan

270 (OAEs) are faint sounds generated by healthy inner ears that provide a window into the study of audit

271 solated from three compartments of the mouse inner ear - the vestibular and cochlear sensory epitheli

272 s encoded within the auditory portion of the inner ear, the cochlea, after propagating down its lengt

273 e as the lens, the olfactory epithelium, the inner ear, the cranial sensory ganglia, and the anterior

274 skin differentiation and carcinogenesis, the inner ear, the lung and the retina.

275 Notably, vestibular sensory organs of the inner ear, the maculae, exhibit a line of polarity rever

276 structure overlying the hearing organ of the inner ear, the organ of Corti.

277 fits for more precise control of delivery of inner ear therapeutic compounds.

278 of sound-induced vibrations in the mammalian inner ear through an active process that involves hair-b

279 The derivation of human inner ear tissue from pluripotent stem cells would enabl

280 mmals owing to the inability of cells in the inner ear to proliferate and replace lost sensory recept

281 tiles shows how minute balance organs in the inner ear transformed at the same time.

282 V2/9 as a novel and atraumatic technique for inner ear transgene delivery in early postnatal mice.

283 , ILDR1 contributes to the ultrastructure of inner ear tTJs.

284 ransmitter to convey excitatory stimuli from inner ear type I vestibular hair cells to postsynaptic c

285 r cells, the mechanosensory receptors of the inner ear, underlie the senses of hearing and balance.

286 The inner ear utricle requires mechanosensory hair cells (HC

287 ation of postsynaptic calyx terminals in the inner ear vestibular organs of contemporary amniotes.

288 into organoids that morphologically resemble inner ear vestibular organs.

289 Delivery of therapeutic compounds to the inner ear via absorption through the round window membra

290 t model of a human middle ear coupled to the inner ear was formulated.

291 TBC1D24 in the mouse inner ear was immunolocalized predominantly to spiral ga

292 s morphology and structure of the pwi larval inner ear was near normal, acoustic startle stimuli evok

293 lian intracochlear anatomy, fixed guinea pig inner ears were imaged as whole temporal bones with coch

294 ntration of iron within the labyrinth of the inner ear, which might indirectly tune a magnetic sensor

295 tonically active synapses in the retina and inner ear with intense vesicle traffic.

296 urements have previously been available from inner ears with intact low-frequency parts.

297 sure to sound that is titrated to stress the inner ear without causing permanent damage would induce

298 l need exists for therapies that protect the inner ear without inhibiting the therapeutic efficacy of

299 In the inner ear, XIRP2 is specifically expressed in hair cells

300 ed with Pax2-Cre, Vangl2 is deleted from the inner ear, yielding planar polarity phenotypes similar t