ライフサイエンスコーパス: endplate

1 the presynaptic fibers to the eventual motor endplate .

2 erse array of molecular targets at the motor endplate.

3 icotinic acetylcholine receptor at the motor endplate.

4 sion without overt degeneration of the motor endplate.

5 on of acetylcholine receptors (AChRs) at the endplate.

6 s) were more frequent near the centre of the endplate.

7 COL13A1 localized to the human muscle motor endplate.

8 lutamate transporter is evident at the motor endplate.

9 erritory they previously occupied within the endplate.

10 causes developmental defects at motor neuron endplates.

11 s at developing, adult, and denervated motor endplates.

12 uscle fibers in the presence of motor neuron endplates.

13 wo different motoneurons at widely separated endplates.

14 nerated nerve terminals still occupied motor endplates.

15 nse in SMA and wild-type normally-innervated endplates.

16 ntration of heparan sulfate proteoglycans at endplates.

17 cells and is required for its clustering at endplates.

18 c strengths were co-regulated at these motor endplates.

19 ion of TSCs at innervated but not denervated endplates.

20 he neuregulin family and is present at motor endplates.

21 ibodies stained neonatal, but not diaphragm, endplates.

22 , EphA8, localizes exclusively to fast motor endplates.

23 and morphological deterioration of the motor endplates.

24 chemistry revealed no abnormalities in motor endplates.

25 level similar to that at denervated original endplates.

26 fic kinase (MuSK) was assessed at denervated endplates.

27 serving agrin on the stability of denervated endplates.

28 d by partial volume averaging with vertebral endplates (173 [27.9%] of 620) and pedicle cortex parall

29 Of 3,300 vertebral endplates, 225 revealed Schmorl nodes: 88 cranial and 13

30 by abnormal signal transmission at the motor endplate, a special synaptic contact between motor axons

31 on of IDC with vacuum phenomena or vertebral endplate abnormalities at any spinal level.

32 The presence of IDC, osteophytes, vertebral endplate abnormalities, and vacuum phenomena was recorde

33 spondylolisthesis, disk degeneration, marrow endplate abnormality (Modic changes), posterior anular h

34 Although the metabolic half-life of muscle endplate acetylcholine receptor (AChR) changes during de

35 c congenital myasthenic syndrome with marked endplate acetylcholine receptor (AChR) deficiency caused

36 region predict reduced rapsyn expression and endplate acetylcholine receptor deficiency.

37 acetylcholine receptor that severely reduce endplate acetylcholine receptor numbers and/or cause kin

38 We used mutations to construct endplate acetylcholine receptors (AChRs) having only one

39 e signalling system coupled to activation of endplate acetylcholine receptors.

40 mutations indicated that there were reduced endplate acetylcholine receptors.

41 ndividuals demonstrate a severe reduction of endplate acetylcholine receptors.

42 in COLQ exon 16 identified in a patient with endplate AChE deficiency causes exclusive skipping of ex

43 Mutations in COLQ cause endplate AChE deficiency in humans.

44 Mutations in COLQ cause endplate AChE deficiency.

45 The consequences of the endplate AChR deficiency are mitigated by persistent exp

46 Endplate studies demonstrated severe endplate AChR deficiency, dispersed endplate regions and

47 The phenotypic consequences are endplate AChR deficiency, simplification of the postsyna

48 DPAGT1 mutations would suggest that reduced endplate AChR due to defective N-linked glycosylation is

49 The M1 transmembrane helix of the muscle endplate AChR is linked to a beta-strand of the extracel

50 tials and endplate potentials, reduced motor endplate AChR number and altered endplate morphology.

51 y exhibited same trend of variation with the endplate after aging.

52 le homeostasis and maintaining neuromuscular endplates after nerve injury.

53 to characterize the 3D morphology changes of endplate and IVD during aging using PPCST.

54 ChR that restores electrical activity at the endplate and rescues the phenotype.

55 es the role of collagens at the human muscle endplate and should facilitate the recognition of this d

56 rvable AChR clusters at the developing motor endplate and that MuSK and AChRs codistribute throughout

57 changes of the IVD and canal network in the endplate and the interaction after aging.

58 A similar analysis between a type 1 endplate and the presence of a disk herniation (PPV, 0.2

59 26; 95% CI: 0.19, 0.34) and between a type 1 endplate and vertebral body spondylolisthesis (PPV, 0.28

60 is might occur if acetylcholine escapes from endplates and binds to extrajunctional fetal-type AChRs

61 Functional connectivity between motor neuron endplates and muscle fibers is confirmed with calcium im

62 the defects that were observed in the motor endplates and muscles of the axJ mice.

63 uggest that osteoclast-initiated porosity of endplates and sensory innervation are potential therapeu

64 at ALG14 is concentrated at the muscle motor endplates and small interfering RNA silencing of ALG14 r

65 ts abrogates sensory innervation into porous endplates and spinal hypersensitivity.

66 nsferase-like immunoreactive en plaque motor endplates and substance P-like immunoreactive, thin and

67 ural and electrophysiological studies of the endplate, and from biochemical studies.

68 s evoke self-contained synaptic responses at endplates, and that these are non-co-operative with resp

69 unctional fold density is reduced at MyoD-/- endplates, and the transition from the fetal (alpha, bet

70 stsynaptic defects including increased motor endplate area and fragmentation were readily observed in

71 endplate size and TSC number, we manipulated endplate area in an androgen-sensitive muscle of the rat

72 was, however, significantly correlated with endplate area in both LA and EDL muscles.

73 No effects on motor endplate area or density were observed across treatment

74 xtensor digitorum longus (EDL) muscle, where endplate area was unaffected by castration or testostero

75 A expression, muscle force production, motor endplate area, and innervation status.

76 hat TSC number increases to match increasing endplate area.

77 e of the typical degenerative changes of the endplate associated with the slow-channel congenital mya

78 lcholine receptors (AChRs) are detected; (b) endplate-associated CGRP declines with muscle denervatio

79 Dach2 and Hdac9 control the expression of endplate-associated genes such as those encoding nicotin

80 ing GABP(alphabeta) transcription factors in endplate-associated myonuclei.

81 t-encoding genes (alpha2betaepsilondelta) in endplate-associated myonuclei.

82 ter denervation, and reorganization of motor endplates at the postsynaptic sites compared with those

83 a focal innervation pattern along a central endplate band.

84 F) mutant demonstrate NMJ defects with wider endplate bands and smaller AChR plaques.

85 e to acetylcholine receptors (AChR) on motor endplates by autoantibody-induced complement attack caus

86 ype in bovine nucleus pulposus and cartilage endplate cells at the gene level.

87 number of these mitotic cells were found at endplates contacted by TSC processes extended from nearb

88 SC processes extended from nearby denervated endplates, contacts known to promote nerve sprouting.

89 fashion: within three days less than 5 % of endplates contained vestiges of nerve terminals.

90 Vertebral endplate contour was analyzed, and abnormalities of the

91 d synaptic strength by prolonging the evoked endplate current (EPC) decay.

92 The temperature dependence of miniature endplate current (MEPC) amplitude (A(c)), 20-80% rise ti

93 bute significantly to the time course of the endplate current decay in these disease conditions.

94 t), (2) reduced amplitude of evoked release (endplate current) and quantal content, (3) age-dependent

95 t any change in quantal amplitude (miniature endplate current), (2) reduced amplitude of evoked relea

96 size of the time integrals of the miniature endplate currents ([integral]MEPCs), measured at the sam

97 termined from endplate potentials (EPPs) and endplate currents (EPCs) in preparations partially block

98 ld reduction in the amplitudes of the evoked endplate currents (EPCs), but normal spontaneous miniatu

99 -fold increase in the frequency of miniature endplate currents (MEPCs) and an increase in NMJ size, b

100 logically, we recorded spontaneous miniature endplate currents (mEPCs) and nerve-evoked EPCs (eEPCs)

101 prolonged the decay phases of the miniature endplate currents (MEPCs) over a broad range.

102 Miniature endplate currents (MEPCs) were recorded at -150 mV with

103 o an increase in the occurrence of miniature endplate currents (mepcs) with abnormally long half-widt

104 The abnormal miniature endplate currents (mEPCs), which have significantly grea

105 ariations in geometry and kinetics relate to endplate currents associated with fast-twitch, slow-twit

106 fast inward currents that resemble miniature endplate currents found at neuromuscular synapses.

107 hypothesis that the altered kinetics of the endplate currents in this disease are attributable to in

108 ressive weakness and decline of amplitude of endplate currents over a period of 14 years.

109 scular junction and the subsequent miniature endplate currents produced at the postsynaptic membrane.

110 that the complete but transient blockade of endplate currents underlies the robust axotomy-like effe

111 t motor terminals after botulinum toxin, but endplate currents were completely blocked for at least s

112 in M3 of the alpha subunit, which attenuates endplate currents, accelerates their decay and causes ab

113 t in the mutant receptor producing prolonged endplate currents, and consequent muscle damage.

114 athy, prolonged and biexponentially decaying endplate currents, and prolonged acetylcholine receptor

115 p, and spine imaging shows several vertebral endplate deformities, but overall preservation of verteb

116 an, 58.5%) and for benign lesions, including endplate degeneration (mean, 52.2%), Schmorl nodes with

117 synaptic remodeling and causes neuromuscular endplate degeneration.

118 marked congenital weakness in the absence of endplate degeneration.

119 or therapeutic intervention to prevent motor endplate degradation following nerve injury.

120 In wild-type mice, the endplates demonstrated time-dependent decreases in area

121 Endplate depolarisation and quantal content per unit are

122 are non-co-operative with respect to overall endplate depolarisation or safety margin for synaptic tr

123 able pi-junctions produced nearly equivalent endplate depolarisations and quantal content per unit ar

124 nt to produce suprathreshold or subthreshold endplate depolarisations.

125 d with the synaptic basal lamina, defects in endplate development and maintenance, or defects in prot

126 ence of Scn8a has been correlated with motor endplate disease (med), in which transient sodium curren

127 anatomical partitioning of muscle fibers and endplate distribution.

128 We demonstrate that shrinkage of motor endplates does not correlate with loss of motor nerve te

129 e localization of AChRs at the neuromuscular endplate during agrin-induced synaptogenesis.

130 the nicotinic acetylcholine receptor at the endplate during synaptic differentiation at the neuromus

131 organ reinnervation is degradation of motor endplates during prolonged denervation.

132 The pathophysiological result is muscle endplate dysfunction and consequent fatigable muscle wea

133 les from MMP3 null mice demonstrated greater endplate efficacy and reinnervation.

134 ly, severe degenerative changes at the motor endplate (endplate myopathy) were apparent by electron m

135 found that TSC number not only increased as endplates enlarged but also decreased when endplates shr

136 uscular junctions were examined by isolating endplate enriched and non-endplate regions identified by

137 nce of cilia on growth plate (GP), cartilage endplate (EP) annulus fibrosus (AF), and nucleus pulposu

138 Asymmetric AChE is concentrated at the endplate (EP), where its collagenic tail anchors it into

139 yndromes (CMSs) stem from genetic defects in endplate (EP)-specific presynaptic, synaptic, and postsy

140 eft, vertebral signal intensity alterations, endplate erosions on T1-weighted MR images, and presence

141 ation of MuSK remains localized to the motor endplate even 14 days after denervation.

142 late with a type 1 change (hereafter, type 1 endplate) for a tear in the annulus fibrosis of the disk

143 How Crk/CrkL regulate neuromuscular endplate formation is not known.

144 Motor endplates formed on the fibers derived from myoblasts of

145 Agrin and MuSK were preserved in endplates from denervated MMP3 null animals.

146 At each of 3,300 endplates from T1 to L5, the presence of Schmorl nodes w

147 Immunofluorescence staining of endplates from the treated animals showed that C9 deposi

148 Analyses of motor endplates from two of the individuals demonstrate a seve

149 to nicotinic acetylcholine receptors of the endplate gutters.

150 Patient endplates, however, are devoid of AChE, suggesting that

151 a-latrotoxin-like effect on the murine motor endplate, i.e. they bring about massive quantal release

152 ile MuSK is normally restricted to the motor endplate in adult muscle, denervation results in its ext

153 rted to guide terminal sprouts to denervated endplates in adult muscles, are necessary for the format

154 neuronal innervation and shrinkage of motor endplates in both diseases.

155 innervation of the porous areas of sclerotic endplates in mice was confirmed.

156 th expression of the fetal gamma-AChR at the endplates in one patient, prolongation of some channel e

157 that the extent of calcium overload of motor endplates in the panel of transgenic mice was influenced

158 tervertebral disc (IVD) and interaction with endplate is essential to elucidate the pathogenesis of I

159 Nerve sprouting at endplates, known as terminal sprouting, is key to normal

160 significant differences in variance of motor endplate length in motor units, which correlated weakly

161 phy of muscle fibres and post-synaptic motor endplates, loss of lower motor neuron cell bodies and de

162 port that synaptic terminals occupying motor endplates made electrically silent by tetrodotoxin and a

163 myotubes from patients with FUS-ALS revealed endplate maturation defects due to intrinsic FUS toxicit

164 ructure of the receptor type found in muscle-endplate membrane and in the muscle-derived electric tis

165 Muscle fiber and endplate morphologies were determined by histochemical m

166 terized receptor area, receptor density, and endplate morphology in denervated plantaris muscles in w

167 icle endocytosis/exocytosis was improved and endplate morphology was restored.

168 duced motor endplate AChR number and altered endplate morphology.

169 Endplate myopathy consists of a combination of ultrastru

170 degenerative changes at the motor endplate (endplate myopathy) were apparent by electron microscopy

171 he severely disabled propositus has advanced endplate myopathy, prolonged and biexponentially decayin

172 opic assessment of the abnormalities seen in endplate myopathy, we found that activated caspases were

173 is selective degeneration of the NMJ termed endplate myopathy.

174 ion or destruction of at least one vertebral endplate (n = 37, 84.1% sensitivity).

175 Neither extension of cement to the endplate nor cement leakage into the disk space has sign

176 in quantal content in inverse proportion to endplate occupancy.

177 at severe denervation (<50% fully innervated endplates) occurs selectively in many vulnerable axial m

178 d terminal branching of motor neurons to the endplate of skeletal muscles, resulting in poor formatio

179 glycosylated dystroglycan in stabilizing the endplate of the NMJ.

180 c nerve terminal with a complex postsynaptic endplate on the muscle surface.

181 a motor nerve terminal to occupy most of an endplate, or to produce a suprathreshold response in ord

182 zebrafish, reminiscent of the neuromuscular endplate pathology seen in patients with DOK7 mutations.

183 The polyneuronally innervated motor endplates (pi-junctions) were identified by vital staini

184 fraction and connectivity of the canals, and endplate porosity and thickness, reached a peak at 4 mon

185 or DuP 697, prevents the delayed increase in endplate potential (EPP) amplitude normally produced by

186 n the kinetics of stretch-induced changes in endplate potential (EPP) amplitude or miniature EPP (mEP

187 ontrol solution), the quantal content of the endplate potential (EPP) depressed more rapidly (approxi

188 depressed nerve control, increased miniature endplate potential (MEPP) amplitude, decreased MEPP freq

189 monstrate an increased spontaneous miniature endplate potential (mEPP) frequency in Nedd4 mutants.

190 ncreases evoked quantal output and miniature endplate potential (MEPP) frequency, again by activating

191 The size of the miniature endplate potential (MEPP) increased 3- or 4-fold in prep

192 aracterized by a reduction in both miniature endplate potential amplitude and AChR abundance accompan

193 Miniature endplate potential amplitude was reduced 3 d after SC ab

194 Miniature endplate potential amplitude, but not frequency, was red

195 s muscle biopsy revealed decreased miniature endplate potential amplitudes, reduced endplate size and

196 t decrease of the amplitude of the miniature endplate potential and no deficiency of the ACh receptor

197 tudy also showed a striking reduction of the endplate potential quantal content, consistent with addi

198 Similar decreases in miniature endplate potential size ([integral]MEPP) followed repeti

199 ing continued, the fraction of the miniature endplate potential voltage-time integrals ( MEPPs) in th

200 nstant of repolarisation of the conditioning endplate potential.

201 nse to nerve stimulation was determined from endplate potentials (EPPs) and endplate currents (EPCs)

202 Similarly, endplate potentials (EPPs) evoked at low frequency were

203 ality between staining/destaining and summed endplate potentials (EPPs) representing total transmitte

204 ll partially or fully occupied and expressed endplate potentials (EPPs).

205 measuring the relative changes of miniature endplate potentials (mEPPs) and voltage responses to ste

206 C on neurosecretion in the form of miniature endplate potentials (MEPPs) were assessed.

207 Miniature endplate potentials (MEPPs) were more frequent near the

208 f spontaneous transmitter release (miniature endplate potentials (MEPPs)) from motor nerve terminals

209 tude or time course of spontaneous miniature endplate potentials (MEPPs).

210 the mean amplitude of spontaneous miniature endplate potentials and bungarotoxin binding.

211 fatigable muscle weakness, reduced miniature endplate potentials and endplate potentials, reduced mot

212 el kinetics, or endplate ultrastructure, but endplate potentials depolarizing the resting potential t

213 tosolic [Ca2+], and reduced the amplitude of endplate potentials evoked after the end of a stimulus t

214 oved synergistic in restoring suprathreshold endplate potentials in mouse diaphragms fully intoxicate

215 the other hand, the mean amplitude of evoked endplate potentials was not decreased, due to an increas

216 The amplitude and rise time of miniature endplate potentials were also increased, but these chang

217 The amplitude and frequency of miniature endplate potentials were reduced, indicating impaired ne

218 ological measurements of ACh secretion (i.e. endplate potentials, EPPs) and the component of the prej

219 ed acetylcholine (ACh) release (reflected as endplate potentials, EPPs) is well described by a simple

220 s, reduced miniature endplate potentials and endplate potentials, reduced motor endplate AChR number

221 s determined by micro-electrode recording of endplate potentials.

222 frequency but not the amplitude of miniature endplate potentials.

223 e most commonly (37 [40%] of 93) a result of endplate proximity, with 32 (34% of 93) caused by low CT

224 It employs their areas on individual endplates quantified by electron microscopy-based analys

225 e is hydrolysed rapidly to choline (Cho), so endplate receptors (AChRs) are exposed to high concentra

226 e is hydrolysed rapidly to choline (Cho), so endplate receptors (AChRs) are exposed to high concentra

227 creased the number of detectable NMJs during endplate recording.

228 animals had fewer active NMJs, detectable by endplate recordings, compared with age-matched wild-type

229 sprouts remain within the boundaries of the endplate region and rarely grow extrasynaptically even i

230 Lrp4 is also expressed in the post-synaptic endplate region of muscles and is required to form neuro

231 ced levels of acetylcholine receptors at the endplate region.

232 mRNA level was elevated 6 fold in the muscle endplate regions and that there were two distinct Calcrl

233 d severe endplate AChR deficiency, dispersed endplate regions and well preserved junctional folds in

234 mined by isolating endplate enriched and non-endplate regions identified by staining for acetylcholin

235 olinesterase (AChE) molecular forms in motor endplate regions of adult Sprague-Dawley rat fast-twitch

236 reduces the activities of all AChE forms in endplate regions of normally innervated and otherwise un

237 ransmitter quanta and appearance of multiple endplate regions on the muscle fiber.

238 uscle, as well as in the corresponding motor endplate regions where high levels of both AChE activity

239 higher levels of CALCRL are localized to the endplate regions.

240 including fiber count/nerve density, muscle endplate reinnervation, compound muscle action potential

241 r communication requires axonal regrowth and endplate reinnervation.

242 d to severely reduced AChR density in mutant endplates relative to controls.

243 emonstrate a critical role for MMP3 in motor endplate remodeling, and reveal a potential target for t

244 nges were attenuated in MMP3 null mice, with endplates retaining their differentiated form.

245 included those with cement extension to the endplate(s) and cement leakage into the disk space(s).

246 included those with cement extension to the endplate(s) but no leakage into the disk space(s), and g

247 ded patients with no cement extension to the endplate(s), group 2 (n=216) included those with cement

248 Findings that were not helpful included endplate sclerosis and erosions, osteophytes, paraspinal

249 Potential imaging discriminators, including endplate sclerosis or erosions, osteophytes, spondylolis

250 gnificantly inhibits LSI-induced porosity of endplates, sensory innervation, and spinal hypersensitiv

251 Pathologically, the motor endplates show focal accumulation of calcium and strikin

252 Recent studies of muscle synapses (endplates) show that there are also terminal Schwann cel

253 The epsilon1245ins18-AChR at the endplate shows abnormally brief activation episodes duri

254 s endplates enlarged but also decreased when endplates shrank.

255 spases were present at between 15 and 57% of endplates, similar to the proportion of endplates with d

256 eight, fiber cross-sectional area, and motor endplate size and density.

257 ature endplate potential amplitudes, reduced endplate size and simplification of secondary synaptic f

258 Furthermore, the relationship between endplate size and TSC number, as defined by the slope of

259 experimentally test the relationship between endplate size and TSC number, we manipulated endplate ar

260 approximately 20% (one cell per junction) as endplate size decreased by 30%.

261 flect a developmental process independent of endplate size or terminal function.

262 cle mass, fiber oxidative capacity, or motor endplate size.

263 weeks and largely precedes the expansion of endplate size.

264 d TSC number in the adult is correlated with endplate size.

265 Fyk, and Src, each formed a complex with the endplate-specific cytoskeletal protein rapsyn.

266 e neuromuscular synapse, innervation induces endplate-specific expression of adult-type nicotinic ace

267 By 10 days, fewer than 20 % of endplates still showed evidence of synaptic activity.

268 , asynchronous synapse withdrawal from motor endplates, strongly resembling neonatal synapse eliminat

269 to degrade agrin, we examined the changes in endplate structure following traumatic nerve injury in M

270 Endplate studies demonstrated severe endplate AChR defic

271 Endplate studies show markedly reduced expression of the

272 ometric observations regarding the vertebral endplates support the concept that Schmorl nodes are cau

273 synaptosomal fractions, suggesting that the endplate swellings may be caused by decreased protein tu

274 ular muscles: those receiving a single motor endplate, termed singly innervated fibers (SIFs), and th

275 ade signaling mechanism located at the motor endplate that enables expression of adult motoneuron exc

276 om innervation would result in many neonatal endplates that are co-innervated by sibling branches.

277 nock-in mice displayed smaller neuromuscular endplates that denervated before motor neuron loss, whic

278 acetylcholine receptors are blocked at motor endplates, the electrical properties of rat motoneurons

279 find MEPPs originating at the margins of the endplate to be strikingly smaller.

280 s, we modified the design to include polymer endplates to interface the DAPS with adjacent vertebrae,

281 ChR, together with the sustained exposure of endplates to serum choline, results in continuous channe

282 Endplate topography and structure were also studied, usi

283 acetylcholine receptor channel kinetics, or endplate ultrastructure, but endplate potentials depolar

284 uscles, terminal Schwann cells at denervated endplates undergo apoptosis.

285 xperiments show calcium release at the motor endplate upon K+ depolarization precisely in these IP3R-

286 dult AChR, but structural development of the endplate was compromised.

287 blocking, the spatial separation between the endplates was estimated to be between 26 and 44 mm.

288 e (LSIVDA), defined as the angle between the endplates, was measured, S1-2 disk morphology was rated

289 days later, receptor regions within a single endplate were divided into differentiated and less organ

290 (P <.001) and fractured (P <.001) vertebral endplates were associated with Schmorl nodes.

291 ced and ultrastructural analyses showed that endplates were intact.

292 Thus, five days after axotomy, 50-90 % of endplates were still partially or fully occupied and exp

293 sts induce sensory innervation in the porous endplates which contributes to spinal hypersensitivity i

294 En grappe endplates, which are normally only found on slow-MyHC ex

295 The PPV of an endplate with a type 1 change (hereafter, type 1 endplat

296 piction of 3D micro-architectural changes of endplate with aging and interaction with IVD remains a t

297 % of endplates, similar to the proportion of endplates with degenerating mitochondria or vacuoles.

298 The cKO mice also had motor endplates with significantly reduced area and thickness.

299 MuSK is necessary for prepatterning of the endplate zone anlage and as a signaling receptor for agr

300 program) are confined to a centrally located endplate zone.