ライフサイエンスコーパス: AChR

1 AChR clusters became fragmented with diminished junction

2 AChR-MG and MuSK-MG subjects displayed distinct gene seg

3 AChRs might provide a valuable proxy to decipher the fun

4 te radiotracer for PET imaging of human M(1) AChR.

5 e extreme subtype specificity of MT7 for M(1)AChR and the mechanism by which it regulates receptor fu

6 ere, we present the crystal structure of M(1)AChR in complex with MT7, a subtype-selective anti-M(1)A

7 mplex with MT7, a subtype-selective anti-M(1)AChR snake venom toxin.

8 , we have converted the selectivity from M(1)AChR toward M(2)AChR, suggesting that the three-finger f

9 iously untested AChR-Ab positive MG sera, 10 AChR-Ab negative MG sera and 5 healthy control sera were

10 b positive sera compared with none of the 10 AChR-Ab negative sera (p=0.0020) inhibited rapsyn-cluste

11 Only 2/11 AChR-Ab positive sera inhibited AChR currents in unclust

12 AChR currents in unclustered AChRs, but 6/11 AChR-Ab positive sera compared with none of the 10 AChR-

13 e agonist's quaternary ammonium (QA) and (2) AChRs respond strongly to ACh because an H-bond position

14 ted the selectivity from M(1)AChR toward M(2)AChR, suggesting that the three-finger fold is a promisi

15 ased visual screen for mutants with abnormal AChR distribution, we isolated the ras suppressor 1 (rsu

16 neurotransmitter receptor for acetylcholine (AChR) display a series of cholesterol consensus domains

17 s reduced fetal AChR currents, but not adult AChR currents, by >80% within 100 s.

18 from desensitization is faster in the adult AChR isoform.

19 overy time constants in both fetal and adult AChRs.

20 from desensitization in both fetal and adult AChRs.

21 unclustered and rapsyn-clustered human adult AChRs in CN21 cells.

22 cell lines expressing either fetal or adult AChRs.

23 the developmental switch from fetal to adult AChRs, as well as how their functions might be modified

24 AChR) response in MG, MHC class II and alpha-AChR subunit as well as chemokines involved in GC develo

25 sduction pathway altered MHC Class II, alpha-AChR, and CXCL13 expression.

26 that estrogens inhibited expression of alpha-AChR and HLA-DR in TECs, suggesting that estrogens may a

27 ta (gamma-AChRs) and alpha1beta1epsilondelta AChRs (epsilon-AChRs) in Xenopus oocytes revealed that P

28 of the M1-M2 linker cysteines of the alpha3 AChR subunit.

29 etylcholine receptor (AChR), the alpha3beta4 AChR and the homomeric alpha1 glycine receptor (GlyR).

30 control the timing of AChR expression in an AChR-less fish background.

31 utant CLASP2 in clusters, but MT capture and AChR cluster size are reduced.

32 ad ocular MG at onset than those with MG and AChR antibodies, although the difference was not statist

33 that the diffusion dynamics of the GM1s and AChRs is uniformly affected by the intracellular ATP lev

34 nd long durations, we find that the GM1s and AChRs share the same dynamic heterogeneity and non-Gauss

35 t the clusters) had little effect on aneural AChR clusters at E13.5, suggesting that SCs may not be n

36 Because anti-AChR antibodies are highly specific for MG and are patho

37 was used in combination with monoclonal anti-AChR antibody labeling of live cells, which induces AChR

38 subsequently induced with a low dose of anti-AChR monoclonal antibody 35.

39 alues were normalized to a pretreatment anti-AChR antibody level of 100% and the mean levels after ea

40 ll responses, decreased levels of serum anti-AChR IgGs, and reduced complement activation at the neur

41 at regulate surface trafficking of assembled AChR and may help prevent surface expression of unassemb

42 ns increased the surface levels of assembled AChR expressed in HEK cells to 138% of wild-type levels.

43 lular peripheral membrane protein that binds AChRs, is essential for synaptic differentiation, but ho

44 linked immunosorbent assay and Western blot; AChR, MuSK, and anti-striated muscle antibodies were det

45 and cytokine production in response to both AChR and control Ags were measured from 3120 T cell libr

46 hronically denervated muscles, in which both AChR stability and recycling are significantly decreased

47 zation time constants were similar with both AChR isoforms but recovery time constants were shorter i

48 We found plectin isoform 1f (P1f) to bridge AChRs and IFs via direct interaction with the AChR-scaff

49 Rapsn is an adapter protein that bridges AChRs to the cytoskeleton and possesses E3 ligase activi

50 tions differentiate as in the wild type, but AChRs assemble into ectopic clusters that progressively

51 scle spindle afferent response to stretch by AChRs in the central region of intrafusal fibres possibl

52 excessive growth of motor axons that bypass AChR clusters.

53 of plasma cells that secrete disease-causing AChR autoantibodies and although thymectomy improves cli

54 These observations also apply to the classic AChR MG phenotype seen in large series.

55 e sera (p=0.0020) inhibited rapsyn-clustered AChR currents, and current inhibition by the AChR-Ab pos

56 The clustered AChR CBA detected antibodies in 38.1% (16 of 42) of RIPA

57 We hypothesised that clustered AChRs would provide a better target for investigating th

58 Patients with antibodies only to clustered AChRs appear to be younger and have milder disease than

59 , patients with antibodies only to clustered AChRs had frequent prepubertal onset (62.5% [median age,

60 AChR antibodies and antibodies to clustered AChRs in 138 patients.

61 ents were tested for antibodies to clustered AChRs, and 42 had a final diagnosis of MG.

62 ition of the competitive and non-competitive AChR blockers d-tubocurarine and alpha-bungarotoxin, res

63 tin-deficient cells rescued both compromised AChR clustering and IF network anchoring.

64 In contrast, AChRs did not recycle at agrin-induced clusters in C2C12

65 f post-transcriptional events in controlling AChR expression in skeletal muscle, and points toward a

66 ing to the plasma membrane and not decreased AChR turnover.

67 significantly altered as a result of delayed AChR expression.

68 Each AChR has two neurotransmitter binding sites located at t

69 rticular abnormalities were unique to either AChR-MG or MuSK-MG, indicating that the repertoires refl

70 Importantly, HuR binds to endogenous AChR beta-subunit transcripts in cultured myotubes and i

71 1 transmembrane helix of the muscle endplate AChR is linked to a beta-strand of the extracellular dom

72 fiber growth; (2) a defective gamma/epsilon-AChR subunit switch, preferentially at synapses on slow

73 ) and alpha1beta1epsilondelta AChRs (epsilon-AChRs) in Xenopus oocytes revealed that PEA selectively

74 ected the rundown of ACh currents in epsilon-AChRs.

75 t, does not require rapsyn because expressed AChRs are visible on the cell membranes of rapsyn-defici

76 R-Ab positive plasmas known to inhibit fetal AChR function in TE671 cells.

77 affinity, and detected the presence of fetal AChR on a number of rhabdomyosarcoma cell lines.

78 However, mAb 131 did not reduce fetal AChR ion channel currents in electrophysiological experi

79 ernal AChR-Ab positive plasmas reduced fetal AChR currents, but not adult AChR currents, by >80% with

80 specific for the gamma-subunit of the fetal AChR to which it bound with sub-nanomolar apparent affin

81 lpha-bungarotoxin binding sites on the fetal AChR, and partially blocked the binding of an antibody (

82 to exchange function between adult and fetal AChRs.

83 behave independently in both adult and fetal AChRs.

84 In fetal AChRs, facilitation of recovery kinetics by rapsyn was i

85 in cells expressing adult compared to fetal AChRs (P < 0.0001).

86 Synaptic sites with fetal AChRs in weanling muscle were ~3% in control and ~40% in

87 neuron innervation determined the sites for AChR clustering, a complete reversal of normal neuromusc

88 We show that at nerve-free AChR clusters induced by agrin in extrasynaptic membrane

89 man recombinant alpha1beta1gammadelta (gamma-AChRs) and alpha1beta1epsilondelta AChRs (epsilon-AChRs)

90 first report of an association between high AChR antibody levels and progression from OMG to general

91 ially expressed cytoplasmic domains of human AChR subunits reduced the development of chronic EAMG in

92 onal EMC members in C. elegans also impaired AChR synthesis and induced the unfolded protein response

93 to manage symptoms, and marginal changes in AChR autoantibody titer.

94 h events cannot fully account for changes in AChR expression following denervation.

95 tiation associated with a drastic deficit in AChR clusters and excessive growth of motor axons that b

96 density, suggesting that SCs are involved in AChR cluster maturation.

97 A reduction in AChR protein was documented in line with the above mRNA

98 ppears that all of the intermediate steps in AChR activation comprise a single, energetically coupled

99 romotes MT capture at clusters and increases AChR cluster size, compared with myotubes that express s

100 otubes cultured on agrin patches that induce AChR clustering in a two-dimensional manner.

101 ld protein, serves as an E3 ligase to induce AChR clustering and NMJ formation, possibly by regulatio

102 ty inhibits rapsyn- as well as agrin-induced AChR clustering in heterologous and muscle cells.

103 and focal vesicle delivery to agrin-induced AChR clusters are also inhibited by microtubule- and act

104 ASP2 regulates AChR density at agrin-induced AChR clusters in cultured myotubes via PI3 kinase acting

105 romotes the formation of large agrin-induced AChR clusters.

106 ession of rapsyn in TE671/CN21 cells induced AChR aggregation and also, surprisingly, shortened recov

107 expression of rapsyn in muscle cells induced AChR clustering and facilitated recovery from desensitiz

108 resulted in smaller and fewer nerve-induced AChR clusters; however, SC ablation at E15.5 reduced ACh

109 tibody labeling of live cells, which induces AChR clustering.

110 ese results show that antibodies can inhibit AChR function rapidly and demonstrate the importance of

111 established EAMG, and that the MDSCs inhibit AChR-specific immune responses at least partially in an

112 Only 2/11 AChR-Ab positive sera inhibited AChR currents in unclustered AChRs, but 6/11 AChR-Ab pos

113 grin in extrasynaptic membrane, internalized AChRs are driven back into the ectopic synaptic clusters

114 s by promoting the recycling of internalized AChRs, which would otherwise be destined for degradation

115 amisole-sensitive acetylcholine receptors (L-AChRs) requires the muscle-secreted scaffolding protein

116 in (SST) interneurons in the mPFC express M1-AChR at higher levels than parvalbumin interneurons.

117 MKII+) interneurons in the mPFC expressed M1-AChR.

118 Moreover, knockdown of M1-AChR in SST interneurons in the mPFC demonstrated that M

119 In mice, viral-mediated knockdown of M1-AChR specifically in GABAergic neurons, but not glutamat

120 -type muscarinic acetylcholine receptors (M1-AChR); however, the cellular mechanisms underlying activ

121 nterneurons in the mPFC demonstrated that M1-AChR expression in these neurons is required for the rap

122 were first demonstrated by applying maternal AChR-Ab positive plasmas known to inhibit fetal AChR fun

123 The maternal AChR-Ab positive plasmas reduced fetal AChR currents, bu

124 eplace GAA to the affected tissue and modify AChR mRNA expression, muscle force production, motor end

125 these and other agonists in adult-type mouse AChRs having a mutation(s) at the transmitter-binding si

126 ons in rats do express Gq-coupled muscarinic AChRs, which appear to have gone undetected in the previ

127 ely express pirenzepine-sensitive muscarinic AChRs.

128 y validated model of the open-channel muscle AChR.

129 chanism for the transformation of the muscle AChR into an inhibitory channel in a clinical context.

130 oteins consisting of CD4 fused to the muscle AChR subunit cytoplasmic loops.

131 Desensitization of human muscle AChRs was investigated using the patch clamp technique t

132 to adult acetylcholine receptors in muscle (AChRs) and the functional impact of AChR clustering by r

133 New AChR clustering is also induced by axon terminals that f

134 n a Golgi-based regulatory step in nicotinic AChR trafficking.

135 he repetitive activation of muscle nicotinic AChRs.

136 and glutamate synaptic release via nicotinic AChRs.

137 R mRNAs due to transcriptional activation of AChR subunit genes.

138 The memory compartment of AChR-MG was further characterized by reduced positive se

139 CLASP2, and LL5beta, for precise delivery of AChR vesicles from the subsynaptic nuclei to the overlyi

140 , increases the size and receptor density of AChR clusters at the NMJ through the delivery of AChRs a

141 for investigating the functional effects of AChR-Abs.

142 t muscle denervation increases expression of AChR mRNAs due to transcriptional activation of AChR sub

143 The extent of AChR recycling depended on the strength of the agrin sti

144 R aggregates, and regulates the formation of AChR.

145 l systems in MT capture and in the fusion of AChR vesicles with the cluster membrane.

146 muscle (AChRs) and the functional impact of AChR clustering by rapsyn are not well studied.

147 in the literature about the implications of AChR antibody levels and progression from OMG to general

148 covery kinetics by rapsyn was independent of AChR clustering.

149 With the delayed induction of AChR expression after an extensive period of AChR-less d

150 his is due to reduced steady-state levels of AChR alpha, delta, epsilon, but not beta subunits rather

151 e also involved in controlling the levels of AChR mRNAs following denervation.

152 bility assays revealed that the half-life of AChR beta-subunit mRNAs was increased in the presence of

153 of CLASP2 play a role in the maintenance of AChR cluster size through the regulated capture and rele

154 2A) gene is essential for the maintenance of AChR clusters both in vivo and in cultured muscle cells.

155 is crucial for formation and maintenance of AChR clusters, postsynaptic NMJ organization, and body l

156 Despite the accepted model of AChR regulation, which implicates transcriptional mechan

157 AChR expression after an extensive period of AChR-less development, paralyzed fish displayed a remark

158 However, in the presence of AChR subunits, rapsyn molecules were targeted to the cel

159 l legs, which correlated with a reduction of AChR protein levels at the neuromuscular junction (appro

160 illin axis participates in the regulation of AChR insertion and removal to control the structure of N

161 and NMJ formation, possibly by regulation of AChR neddylation.

162 med deep sequencing of the BCR repertoire of AChR-MG, MuSK-MG, and healthy subjects to generate appro

163 ne characteristics, OMG symptoms, results of AChR antibody testing, and progression time to generaliz

164 We demonstrate a higher sensitivity of AChR antibody testing than previously reported in the la

165 at the NMJ and how this controls the size of AChR clusters are not yet understood.

166 turn, causes an increase in the stability of AChR beta-subunit mRNAs in denervated muscle.

167 y leading to an increase in the stability of AChR beta-subunit transcripts.

168 in which we can freely control the timing of AChR expression in an AChR-less fish background.

169 ubunits rather than altered transcription of AChR-subunit RNA.

170 (ARE) in the 3'-untranslated region (UTR) of AChR beta-subunit mRNA.

171 ase and consequently for dense clustering of AChRs, we hypothesized that reduced levels of Dok-7 incr

172 As a consequence, the synaptic content of AChRs is reduced.

173 clusters at the NMJ through the delivery of AChRs and that this is regulated by a pathway involving

174 chanisms that underlie the focal delivery of AChRs to the adult NMJ are not yet understood in detail.

175 ulate the metabolic stability and density of AChRs by promoting the recycling of internalized AChRs,

176 the maintenance of high-level expression of AChRs.

177 indings indicate that the focal insertion of AChRs into the postsynaptic membrane is regulated by sta

178 in vivo reduces the density and insertion of AChRs into the postsynaptic membrane.

179 ubes, neural agrin promotes the recycling of AChRs and thereby increases their metabolic stability.

180 sults provide evidence for an active role of AChRs in the targeting of rapsyn to the NMJ in vivo SIGN

181 the synapse, as well as the stabilization of AChRs.

182 In contrast, the targeting of AChRs to the cell membrane does not require rapsyn.

183 The targeting of AChRs to the cell membrane, in contrast, does not requir

184 atellite cells (Pax7-Cre/cKO), uncoupling of AChRs from IFs was shown to lead to loss of postsynaptic

185 henia gravis (MG), but is usually studied on AChRs expressed in cell lines, rather than tightly clust

186 ChR), muscle-specific kinase (MuSK) or other AChR-related proteins in the postsynaptic muscle membran

187 Among the 223 participants, AChR antibody testing results were positive in 158 parti

188 cetylcholine receptor autoantibody-positive (AChR+) generalized MG.

189 impaired synaptic structure as postsynaptic AChR clusters and their associated postsynaptic scaffold

190 m in response to the absence of postsynaptic AChRs, may underlie symptoms of neuromuscular diseases c

191 in mice lead nerve terminals to prepatterned AChRs.

192 ion in HEK293 cells expressing a delta-R375H AChR mutant that did not form clusters in C2C12 myotubes

193 durability of response and/or relapse rate, AChR autoantibody levels, adverse effects, and inflammat

194 teins, primarily the acetylcholine receptor (AChR) and inhibit signaling at the neuromuscular junctio

195 ction, primarily the acetylcholine receptor (AChR) and the muscle-specific kinase.

196 improve detection of acetylcholine receptor (AChR) antibodies in patients with myasthenia gravis (MG)

197 iated mainly by anti-acetylcholine receptor (AChR) antibodies.

198 The sensitivity of acetylcholine receptor (AChR) antibody testing is thought to be lower in ocular

199 hat govern nicotinic acetylcholine receptor (AChR) assembly and trafficking are poorly defined, and t

200 ressive denervation, acetylcholine receptor (AChR) cluster fragmentation, and neuromuscular dysfuncti

201 ich is essential for acetylcholine receptor (AChR) clustering and NMJ (neuromuscular junction) format

202 12 cell line reduced acetylcholine receptor (AChR) clustering during myotube differentiation.

203 kL complex, regulate acetylcholine receptor (AChR) clustering in vitro, and are localized at synapses

204 rotein necessary for acetylcholine receptor (AChR) clustering; and expression of rapsyn in muscles at

205 rol the stability of acetylcholine receptor (AChR) clusters on the surface of cultured myotubes.

206 reduces the size of acetylcholine receptor (AChR) clusters.

207 he expression of the acetylcholine receptor (AChR) epsilon subunit gene mRNA in both muscles.

208 Direct inhibition of acetylcholine receptor (AChR) function by autoantibodies (Abs) is considered a r

209 A muscle acetylcholine receptor (AChR) has two neurotransmitter binding sites located in

210 mation of the muscle acetylcholine receptor (AChR) into an inhibitory channel.

211 high density of the acetylcholine receptor (AChR) is the hallmark of the neuromuscular junction.

212 ies to the nicotinic acetylcholine receptor (AChR) or to muscle-specific tyrosine kinase (MuSK).

213 nes involved in anti-acetylcholine receptor (AChR) response in MG, MHC class II and alpha-AChR subuni

214 ed expression of the acetylcholine receptor (AChR) subunit, ACR-12::GFP.

215 uscle-type nicotinic acetylcholine receptor (AChR), although there are aspects of normal NMJ developm

216 tibodies against the acetylcholine receptor (AChR), muscle-specific kinase (MuSK) or other AChR-relat

217 e human adult-muscle acetylcholine receptor (AChR), the alpha3beta4 AChR and the homomeric alpha1 gly

218 the muscle nicotinic acetylcholine receptor (AChR), we have recently hypothesized that the conformati

219 plasma membrane, and acetylcholine receptor (AChR), which is a well-characterized ion channel.

220 brane protein, as an acetylcholine receptor (AChR)-associated protein, and we provide evidence that v

221 ce showed suppressed acetylcholine receptor (AChR)-specific T cell responses, decreased levels of ser

222 directed toward the acetylcholine receptor (AChR).

223 bodies targeting the acetylcholine receptor (AChR-MG) or muscle specific kinase (MuSK-MG).

224 y it was shown that acetylcholine receptors (AChR) are concentrated in the equatorial region at the c

225 e the clustering of acetylcholine receptors (AChRs) and increase their metabolic stability in the mus

226 etal muscles, where acetylcholine receptors (AChRs) are concentrated to control muscle contraction.

227 Acetylcholine receptors (AChRs) are heteromeric membrane proteins essential for n

228 ) traps and anchors acetylcholine receptors (AChRs) at high density at the synapse.

229 r the clustering of acetylcholine receptors (AChRs) at postsynaptic sites.

230 r the clustering of acetylcholine receptors (AChRs) at synaptic sites between mammalian motor neurons

231 Neuromuscular acetylcholine receptors (AChRs) have two transmitter binding sites: at alpha-delt

232 construct endplate acetylcholine receptors (AChRs) having only one functional neurotransmitter-bindi

233 ion of postsynaptic acetylcholine receptors (AChRs) impacts presynaptic release by establishing a gen

234 on of extrasynaptic acetylcholine receptors (AChRs) in Caenorhabditis elegans muscle cells.

235 Live imaging of acetylcholine receptors (AChRs) in cultured myotubes differentiated ex vivo from

236 ts in expression of acetylcholine receptors (AChRs) in skeletal muscle that occur even in the absence

237 the high density of acetylcholine receptors (AChRs) in the postsynaptic muscle membrane.

238 dult-type nicotinic acetylcholine receptors (AChRs) mediate signalling at mature neuromuscular juncti

239 Nicotinic acetylcholine receptors (AChRs) switch on/off to generate transient membrane curr

240 to muscle nicotinic acetylcholine receptors (AChRs) that impair neuromuscular transmission, thereby c

241 rough activation of acetylcholine receptors (AChRs), (2) enhances glutamatergic synaptic transmission

242 nsmitter receptors, acetylcholine receptors (AChRs), to the postsynaptic membrane, ensuring for relia

243 the distribution of acetylcholine receptors (AChRs).

244 unction, especially acetylcholine receptors (AChRs).

245 e redistribution of acetylcholine receptors (AChRs).

246 out the function of different ACh receptors (AChRs).

247 dly to choline (Cho), so endplate receptors (AChRs) are exposed to high concentrations of both of the

248 dly to choline (Cho), so endplate receptors (AChRs) are exposed to high concentrations of both of the

249 adult-type muscle mouse nicotinic receptors (AChRs) having mutations of agonist binding site amino ac

250 adrenergic receptor, muscarinic-2 receptors, AChR-nicotinic ganglionic alpha-3 receptors and calcium

251 ity, agrin maintained the amount of recycled AChRs at agrin-induced clusters at a level similar to th

252 sters; however, SC ablation at E15.5 reduced AChR cluster size but had no effect on cluster density,

253 of GFPT1 expression both resulted in reduced AChR cell-surface expression.

254 romuscular diseases characterized by reduced AChRs, such as myasthenia gravis.

255 effective option in patients with refractory AChR+ MG, who were observed to have a durable response a

256 post-transcriptional events indeed regulate AChR beta-subunit mRNAs in response to denervation.

257 e plus end-tracking protein CLASP2 regulates AChR density at agrin-induced AChR clusters in cultured

258 of post-transcriptional events in regulating AChR beta-subunit mRNAs and point toward a central role

259 ibility to passive transfer MG, by rendering AChR clusters less resistant to the autoantibody attack.

260 genita which can be caused by fetal-specific AChR-blocking autoantibodies.

261 RIPA) and CBA were used to test for standard AChR antibodies and antibodies to clustered AChRs in 138

262 These results indicate that AChRs play a critical role in the insertion and/or assoc

263 ry from desensitization is determined by the AChR isoform-specific cytoplasmic M3-M4 domain.

264 AChR currents, and current inhibition by the AChR-Ab positive sera was greater when the AChRs were cl

265 luciferase reporter construct containing the AChR beta-subunit 3'UTR, caused an increase in luciferas

266 However, when the coiled-coil domain (the AChR-binding domain of rapsyn) is deleted, rapsyn fails

267 Performing patch-clamp experiments, the AChR was found to be converted into chloride conductance

268 e amount of energy that is available for the AChR conformational change provided by different, struct

269 es have shown that the alpha2-subunit of the AChR (Chrna2) is expressed in the basal forebrain, in th

270 None of the AChR kink mutations had a measureable effect on agonist

271 Some of the AChR loss that follows denervation is correlated with fa

272 y conduction-catalyzing conformations of the AChR's selectivity-filter glutamates.

273 tibody (mAb 637) to the alpha-subunit of the AChR, suggesting that both antibodies bind at or near on

274 ChRs and IFs via direct interaction with the AChR-scaffolding protein rapsyn in an isoform-specific m

275 ight be modified by rapsyn that clusters the AChRs.

276 e AChR-Ab positive sera was greater when the AChRs were clustered (p=0.0385).

277 To address the function of these AChRs, single unit sensory afferents were recorded from

278 Interestingly, rapsyn, a protein critical to AChR clustering, was reduced in mutant muscle cells; and

279 of both IFN-gamma and IL-17, in response to AChR, was also restricted to the CCR6(+) memory T cell c

280 rom MG patients proliferating in response to AChR-derived peptides was significantly higher than that

281 vide evidence that vezatin binds directly to AChRs.

282 traightening of the M1 proline kink triggers AChR desensitization.

283 ature neuromuscular junctions and fetal-type AChRs are necessary for proper synapse development.

284 sera inhibited AChR currents in unclustered AChRs, but 6/11 AChR-Ab positive sera compared with none

285 Eleven previously untested AChR-Ab positive MG sera, 10 AChR-Ab negative MG sera an

286 wn to increase around the time of birth when AChRs cluster at the developing neuromuscular junctions.

287 uromuscular junction (NMJ) development where AChR clustering precedes innervation.

288 In cultured myoblasts (in which AChRs are absent), myristoylated WT rapsyn mostly locali

289 6 in skeletal muscle cells, colocalizes with AChR aggregates, and regulates the formation of AChR.

290 Immunization with AChR cytoplasmic domains in adjuvant is promising as a s

291 had dSNMG, 19 of 201 (9.5%) who had MG with AChR antibodies (significantly lower than those with dSN

292 8 (15.2%) had dSNMG, 201 (80.4%) had MG with AChR antibodies, and 11 (4.4%) had MG with MuSK antibodi

293 ibodies, compared with those who had MG with AChR antibodies, more frequently had mild forms at onset

294 case series study included 16 patients with AChR+ MG referred to an MG clinic from January 1, 2007,

295 The majority of patients under 50 y with AChR autoantibody MG have thymic lymphofollicular hyperp

296 the cell surface and formed aggregates with AChRs.

297 ) is deleted, rapsyn fails to associate with AChRs at NMJs of living mice.

298 We screened for proteins that coisolate with AChRs in a Rapsyn-dependent manner and show that microtu

299 to the cell surface via its interaction with AChRs.

300 Thus, rapsyn interactions with AChRs lead not only to clustering, but also to a cluster