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 erritory they previously occupied within the endplate.

9 wo different motoneurons at widely separated endplates.

10 nerated nerve terminals still occupied motor endplates.

11 ntration of heparan sulfate proteoglycans at endplates.

12 cells and is required for its clustering at endplates.

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

14 ion of TSCs at innervated but not denervated endplates.

15 he neuregulin family and is present at motor endplates.

16 , EphA8, localizes exclusively to fast motor endplates.

17 ibodies stained neonatal, but not diaphragm, endplates.

18 d occurs homogeneously throughout individual endplates.

19 hragm endplates weakly stained rare neonatal endplates.

20 and morphological deterioration of the motor endplates.

21 chemistry revealed no abnormalities in motor endplates.

22 level similar to that at denervated original endplates.

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

24 serving agrin on the stability of denervated endplates.

25 causes developmental defects at motor neuron endplates.

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

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

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

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

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

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

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

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

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

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

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

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

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

39 mutations indicated that there were reduced endplate acetylcholine receptors.

40 ndividuals demonstrate a severe reduction of endplate acetylcholine receptors.

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

42 Mutations in COLQ cause endplate AChE deficiency in humans.

43 Mutations in COLQ cause endplate AChE deficiency.

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

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

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

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

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

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

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

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

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

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

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

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

56 26; 95% CI: 0.19, 0.34) and between a type 1 endplate and vertebral body spondylolisthesis (PPV, 0.28

57 (EPPs) were released over the same length of endplate and with the same relative probabilities at dif

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

59 All en grappe endplates and certain en plaque endplates of EOM are the

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

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

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

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

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

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

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

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

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

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

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

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

72 hat TSC number increases to match increasing endplate area.

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

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

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

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

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

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

79 to remain in their original location on the endplate ('attached' one-bouton synapse).

80 a focal innervation pattern along a central endplate band.

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

82 duced molecular reorganizations at the motor endplate, but the mechanism of action of agrin remains p

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

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

85 AMECH opens endplate channels for about half of the average duration

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

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

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

89 Vertebral endplate contour was analyzed, and abnormalities of the

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

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

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

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

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

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

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

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

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

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

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

101 We recorded miniature endplate currents (mEPCs) using simultaneous voltage cla

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 efore when AMECH is present in a quantum the endplate currents decay more rapidly.

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

143 Motor endplates formed on the fibers derived from myoblasts of

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

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

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

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

148 to nicotinic acetylcholine receptors of the endplate gutters.

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

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

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

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

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

154 eath of terminal Schwann cells at denervated endplates in neonatal muscles.

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

156 ted at synaptic junctions in brain and motor endplates in skeletal muscle.

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

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

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

162 Muscle fiber and endplate morphologies were determined by histochemical m

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

164 duced motor endplate AChR number and altered endplate morphology.

165 ime and a severe CMS characterized by severe endplate myopathy and extensive remodeling of the postsy

166 Endplate myopathy consists of a combination of ultrastru

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

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

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

170 is selective degeneration of the NMJ termed endplate myopathy.

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

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

173 in quantal content in inverse proportion to endplate occupancy.

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

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

176 glycosylated dystroglycan in stabilizing the endplate of the NMJ.

177 ll en grappe endplates and certain en plaque endplates of EOM are the only mature endplates that coex

178 ding radially at 25 nm x ms(-1) (rapid, from endplate omega figure appearance) or 0.275 nm x ms(-1) (

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

199 nstant of repolarisation of the conditioning endplate potential.

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

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

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

203 Spontaneous MEPPs and uniquantal endplate potentials (EPPs) were released over the same l

204 mplitude; extracellular stimulation elicited endplate potentials (EPPs) which resembled MEPPs.

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

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

207 Boutons produced spontaneous miniature endplate potentials (MEPPs) of nearly normal amplitude;

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

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

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

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

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

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

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

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

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

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

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

219 On average, 63% of the endplate potentials were also seen in both recordings.

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

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

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

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

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

225 frequency but not the amplitude of miniature endplate potentials.

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

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

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

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 r communication requires axonal regrowth and endplate reinnervation.

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

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

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

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

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

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

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

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

249 eptor tyrosine kinase localized to the motor endplate, seemingly well positioned to receive a key ner

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

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

252 s endplates enlarged but also decreased when endplates shrank.

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

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

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

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

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

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

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

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

261 weeks and largely precedes the expansion of endplate size.

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

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

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

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

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

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

268 Endplate studies demonstrated severe endplate AChR defic

269 Endplate studies show markedly reduced expression of the

270 The concentration of nNOS at the muscle endplate suggests NO could serve as a messenger pre- and

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

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

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

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

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

276 plaque endplates of EOM are the only mature endplates that coexpress the adult and fetal AChR isofor

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

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

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

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

281 Endplate topography and structure were also studied, usi

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

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

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

285 nts were recorded from rat lumbrical muscles endplates using low concentrations of ACh and 2.5-100 mi

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 silon antibodies intensely stained diaphragm endplates weakly stained rare neonatal endplates.

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

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

292 ced and ultrastructural analyses showed that endplates were intact.

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

294 but more clusters were detected close to the endplate where the endogenous level of NaChs was higher.

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

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

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

298 % of endplates, similar to the proportion of endplates with degenerating mitochondria or vacuoles.

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.