ライフサイエンスコーパス: musk

1 he muscle specific receptor tyrosine kinase (MUSK).

2 protein 4 (Lrp4) and muscle-specific kinase (MuSK).

3 he muscle-specific receptor tyrosine kinase (MuSK).

4 AChR) or to muscle-specific tyrosine kinase (MuSK).

5 s have antibodies to muscle-specific kinase (MuSK).

6 markers of neuromuscular dysfunction (CHRNA1/MUSK).

7 eceptor for muscle-specific tyrosine kinase (MuSK).

8 ne complex is required for the activation of MuSK.

9 ator to promote association between Lrp4 and MuSK.

10 eptide representing the Dok7-binding site on MuSK.

11 n essential intracellular binding protein of MuSK.

12 27V are both located in the kinase domain of MuSK.

13 the membrane and formation of a complex with MuSK.

14 ed uniformly in mice lacking either agrin or MuSK.

15 for proper folding of Ig1 and processing of MuSK.

16 ecessary and sufficient for association with MuSK.

17 in that acts by binding to LRP4 to stimulate MuSK.

18 develop antibodies against agrin, LRP4, and MuSK.

19 s to neural Agrin by binding and stimulating MuSK.

20 of how signals are transduced from agrin to MuSK.

21 ytoplasmic domain of muscle-specific kinase (MuSK), a major component of the agrin receptor.

22 itter receptor, (ii) muscle-specific kinase (MuSK), a receptor tyrosine kinase essential for the form

23 nction downstream of muscle-specific kinase (MuSK), a receptor tyrosine kinase expressed in skeletal

24 receptor family, and muscle-specific kinase (MuSK), a receptor tyrosine kinase.

25 scle proteins: LRP4, the receptor for Agrin; MuSK, a receptor tyrosine kinase (RTK); and Dok7 (or Dok

26 uromuscular synapse formation by stimulating MuSK, a receptor tyrosine kinase expressed in skeletal m

27 Agrin activates MuSK, a receptor tyrosine kinase expressed in skeletal m

28 ve distinct mouse cDNAs encoding isoforms of MuSK, a receptor tyrosine kinase required for the develo

29 This muscle prepattern requires MuSK, a receptor tyrosine kinase that is essential for s

30 MuSK, a receptor tyrosine kinase that is expressed in sk

31 ors requires Lrp4, a LDLR family member, and MuSK, a receptor tyrosine kinase.

32 rin, a factor released from motoneurons, and MuSK, a transmembrane tyrosine kinase that is activated

33 These papers substantially reshape the agrin-MuSK-ACh hypothesis of neuromuscular synaptogenesis.

34 surface LRP4 levels, inhibited agrin-induced MuSK activation and AChR clustering, and activated compl

35 Disruption of lipid rafts inhibits MuSK activation and downstream signaling and AChR cluste

36 rin, forms a complex with MuSK, and mediates MuSK activation by Agrin.

37 e structure provides the molecular basis for MuSK activation by Dok7 and for rationalizing several Do

38 is responsible for transducing signals from MuSK activation to AChR clustering, culminating in cross

39 trate that Lrp4 is necessary, independent of MuSK activation, for presynaptic differentiation in vivo

40 osphorylation of the AChR, without affecting MuSK activation.

41 ophobic patch are critical for agrin-induced MuSK activation.

42 Furthermore, we found that MuSK also plays an important role in mediating hippocamp

43 Hippocampal disruption of MuSK also prevents the learning-dependent induction of b

44 agrin-induced phosphorylation/activation of MuSK and activation of Rac-1.

45 s accompanied by tyrosine phosphorylation of MuSK and betaAChR.

46 and that the combinatorial function of UnpFL/MuSK and dystroglycan generates diverse patterns of vert

47 pathway, Tid1, which directly interacts with MuSK and is responsible for transducing signals from MuS

48 While the agrin receptor components MuSK and Lrp4 were below detection level in neuron popul

49 like domain of MuSK, prevent binding between MuSK and Lrp4, and inhibit Agrin-stimulated MuSK phospho

50 ceptor clusters, and increased expression of MuSK and Lrp4, two cell surface receptors required for N

51 esterase, choline acetyltransferase, rapsyn, MuSK and Na(v)1.4.

52 to neuromuscular synapse formation, such as MuSK and nAChRs, are induced before muscle innervation o

53 PDZRN3 binds to MuSK and promotes its ubiquitination.

54 which promotes association between Lrp4 and MuSK and stimulates MuSK kinase activity.

55 How Agrin activates MuSK and stimulates synaptic differentiation is not know

56 napse formation by binding agrin, activating MuSK and stimulating postsynaptic differentiation, and f

57 ynapse formation can occur in the absence of MuSK and that the combinatorial function of UnpFL/MuSK a

58 Biglycan binds MuSK and the levels of this receptor tyrosine kinase are

59 e find that muscle-specific receptor kinase (MuSK) and its putative ligand Wnt11r are crucial for res

60 l hour lag, requires muscle-specific kinase (MuSK), and is accompanied by tyrosine phosphorylation of

61 immunosorbent assay and Western blot; AChR, MuSK, and anti-striated muscle antibodies were detected

62 Mutations have been identified in agrin, MuSK, and LRP4 in patients with congenital myasthenic sy

63 s a receptor for Agrin, forms a complex with MuSK, and mediates MuSK activation by Agrin.

64 ce share similar mechanisms, requiring Lrp4, MuSK, and neuronal Agrin but not the MuSK Fz-like domain

65 te that Dok-7 also functions downstream from MuSK, and we identify the proteins that are recruited to

66 epresentative of antibacterial agents, nitro-musks, and surfactants, respectively.

67 MG with MuSK antibodies (MuSK-MG) is often associated with persi

68 in neuromuscular transmission failure since MuSK antibodies alter neuromuscular junction morphology

69 However, patients without AChR or MuSK antibodies appear to be similar to those with AChR

70 MuSK antibodies are found in a variable proportion of AC

71 The patients with neither AChR nor MuSK antibodies are often called seronegative (seronegat

72 her immune components, suggesting that these MuSK antibodies cause disease by directly interfering wi

73 MuSK antibodies define a form of myasthenia gravis that

74 MuSK antibodies per se may predispose to muscle thinning

75 imilar approach was used to demonstrate that MuSK antibodies, although mainly IgG4, were partially Ig

76 h AChR antibodies, and 11 (4.4%) had MG with MuSK antibodies.

77 y may be helpful in patients without AChR or MuSK antibodies.

78 s are double-negative for anti-AChR and anti-MuSK antibodies.

79 23.7%; P = .02), and 0 of 11 who had MG with MuSK antibodies; 0 of 29 controls had cortactin antibodi

80 , and in others anti-muscle-specific kinase (MuSK) antibodies that show pathogenic effects in vivo.

81 de studies confirm three major phenotypes in MuSK antibody positive myasthenia gravis (MMG) patients:

82 Antibodies to muscle specific kinase [MuSK; MuSK antibody positive myasthenia gravis (MuSK-MG)] make

83 MuSK antibody positive patients represent a unique subse

84 certain how muscle specific tyrosine kinase (MuSK) antibody positive myasthenia gravis results in neu

85 reatment of muscle-specific tyrosine kinase (MuSK) antibody positive myasthenia gravis will be review

86 he muscle-specific receptor tyrosine kinase (MuSK) are essential for the acetylcholine receptor (AChR

87 linesterase, and the muscle-specific kinase, MuSK, are expressed selectively by a small number of myo

88 which bind the Frizzled (Fz)-like domain in MuSK, are required for prepatterning, suggesting that Wn

89 MuSK-associated proteins such as Dok7, LRP4, and Wnt11r

90 Here, we demonstrated that MuSK became rapidly internalized in response to agrin, w

91 e we show that pathogenic IgG4 antibodies to MuSK bind to a structural epitope in the first Ig-like d

92 kinase expressed in skeletal muscle, and the MuSK binding protein Dok-7.

93 aveolin-3 is a novel muscle-specific kinase (MuSK) binding protein and that altered nAChR clustering

94 LDLR family member that forms a complex with MuSK, binds neural agrin and stimulates MuSK kinase acti

95 owed that myogenin is necessary for not only MuSK but also nAChR gene regulation by muscle activity.

96 ) have antibodies to muscle specific kinase (MuSK), but a full understanding of their frequency, the

97 ted to RTKs, Dok7 is not only a substrate of MuSK, but also an activator of MuSK's kinase activity.

98 Activation of MuSK by agrin, a neuronally derived heparan-sulfate prot

99 in cultured myotubes show that regulation of MuSK by PDZRN3 plays an important role in MuSK-mediated

100 Regulation of cell surface levels of MuSK by PDZRN3 requires the ubiquitin ligase domain and

101 dings demonstrate that missense mutations in MUSK can result in a severe form of CMS and indicate tha

102 nd sequences of these exons are conserved in MuSK cDNAs from different species.

103 SK's association with itself is specific, as MuSK clusters at the cell surface are segregated from cl

104 recruit another receptor tyrosine kinase to MuSK clusters.

105 method for the determination of 9 synthetic musk compounds in seafood products by combining the quic

106 The method was applied to quantify musk compounds in seafood products from the European sou

107 The ectodomain of MuSK comprises three immunoglobulin-like domains and a c

108 Dysfunction of MuSK CRD in patients has been recently associated with t

109 Together, our data reveal that MuSK CRD is critical for NMJ formation and plays an unsu

110 Expression studies in MuSK deficient myotubes revealed that A727V, which is lo

111 We propose that a Wnt11r-MuSK dependent, PCP-like pathway restricts neural crest

112 ling endosomes and that this localization is MuSK dependent.

113 ence for cooperative and partially redundant MuSK-dependent functions of basement membrane in AChR as

114 hat this prepatterning of AChRs, via a novel MuSK-dependent Wnt pathway, may guide motor axons to the

115 tivation of ERK1/2 in myotubes that was Lrp4/MuSK-dependent.

116 ese IgG4 antibodies have no direct effect on MuSK dimerization or MuSK internalization.

117 Once recruited to MuSK, Dok-7 directly stimulates MuSK kinase activity.

118 proteins that associate with the initiating MuSK/Dok-7/Crk/CrkL complex, regulate acetylcholine rece

119 of NSF expression and NSF mutation attenuate MuSK downstream signaling.

120 eta) expression, suggesting that the role of MuSK during memory consolidation critically involves the

121 noglobulin-like domains (Ig1 and Ig2) of the MuSK ectodomain at 2.2 A resolution.

122 that Wnt11r binds to the zebrafish unplugged/MuSK ectodomain to organize this central muscle zone.

123 leimide sensitive factor (NSF) in regulating MuSK endocytosis and subsequent signaling in response to

124 We report on a 60-bp MuSK enhancer that confers promoter regulation by muscle

125 usk is prepatterned in muscle and that early Musk expression in developing myotubes is sufficient to

126 y with MyoD, a myogenic factor essential for MuSK expression in muscle cells.

127 We also provide evidence that MuSK expression in the hippocampus is required for memor

128 MuSK expression is tightly regulated during development,

129 ere we identified a novel mechanism by which MuSK expression may be regulated.

130 We further show that ectopic Musk expression promotes ectopic synapse formation, indi

131 In addition, ectopic Musk expression stimulates synapse formation in the abse

132 mportant role for CREB1 in the regulation of MuSK expression.

133 uggesting a role for CREB1 in attenuation of MuSK expression.

134 on source helped to distinguish two isomeric musk fragrances by means of different ionization behavio

135 The results show that biocides, musk fragrances, and other personal care products were t

136 , biocides, additives, corrosion inhibitors, musk fragrances, UV light stabilizers, and industrial ch

137 s cause disease by directly interfering with MuSK function.

138 Here, we report the crystal structure of the MuSK Fz-CRD at 2.1 A resolution.

139 ast, prepatterning in zebrafish requires the MuSK Fz-like domain but not Lrp4.

140 g Lrp4, MuSK, and neuronal Agrin but not the MuSK Fz-like domain or Wnt production from muscle.

141 patterning in mice requires Lrp4 but not the MuSK Fz-like domain.

142 Two commonly used synthetic polycyclic musks, galaxolide (HHCB) and tonalide (AHTN), were selec

143 ike element in the 5'-flanking region of the MuSK gene binds to CREB1 (CRE-binding protein 1).

144 Agrin stimulates synapse-specific MuSK gene expression by activating GABP(alphabeta) trans

145 mechanism by which muscle activity regulates MuSK gene expression is not known.

146 MuSK gene expression is regulated by nerve-derived agrin

147 denervated muscle suppressed Mgn, nAChR, and MuSK gene induction, whereas Dach2 knockdown induced Mgn

148 Muscle-specific tyrosine kinase receptor (MuSK) has been believed to be mainly expressed and funct

149 ipoprotein receptor-related protein 4 (LRP4)/MuSK, has been described as an antigen in dSNMG.

150 he muscle-specific receptor tyrosine kinase, MuSK, have critical roles in synapse-specific transcript

151 hR) and a kinase critical for NMJ formation, MuSK; however, a proportion of MG patients are double-ne

152 The structure reveals that MuSK Ig1 and Ig2 are Ig-like domains of the I-set subfam

153 LRP4 also forms a complex with MuSK in a manner that is stimulated by agrin.

154 ere, we demonstrate that the CRD deletion of MuSK in mice caused profound defects of both muscle prep

155 n that is thought to act in cis to stimulate MuSK in muscle fibers for postsynaptic differentiation.

156 uce additional complexity to the activity of MuSK in muscle.

157 g a novel, evolutionarily conserved role for MuSK in neural crest migration.

158 emonstrating that the postsynaptic cell, and MuSK in particular, has a potent role in regulating the

159 ped procedure was applied to determine nitro musks in environmental water samples and was demonstrate

160 sis, was developed for the analysis of nitro musks in environmental water samples.

161 or the determination of synthetic polycyclic musks in oyster samples by using one-step microwave-assi

162 DEP), dibutyl phthalate (DBP), and synthetic musks in the gas phase and for DEHP, DiBP, DBP, and DINP

163 activation of the receptor tyrosine kinase, MuSK, in the postsynaptic membrane.

164 suggesting that association between Lrp4 and MuSK, independent of additional ligands, initiates prepa

165 e N-terminal half of Tid1 induced agrin- and MuSK-independent phosphorylation and clustering of AChRs

166 However, the physiological role of Wnt-MuSK interaction in NMJ formation and function remains t

167 MuSK interacts with the Wnt morphogens, through its Friz

168 ave no direct effect on MuSK dimerization or MuSK internalization.

169 equently, a muscle-specific tyrosine kinase (MuSK) involved in AChR clustering.

170 MuSK is activated by agrin, a neuron-derived heparan sul

171 We demonstrate that UnpFL/MuSK is critical for the assembly of focal synapses in z

172 type I receptor-like protein tyrosine kinase MuSK is essential for the neuromuscular junction formati

173 Here we show that MuSK is expressed in the brain, particularly in neurons,

174 The receptor tyrosine kinase MuSK is indispensable for nerve-muscle synapse formation

175 MuSK is necessary for prepatterning of the endplate zone

176 The muscle-specific kinase MuSK is one of the key molecules orchestrating neuromusc

177 Here we show that expression of Musk is prepatterned in muscle and that early Musk expre

178 Muscle-specific kinase (MuSK) is a receptor tyrosine kinase expressed exclusivel

179 Muscle-specific kinase (MuSK) is an essential receptor tyrosine kinase for the e

180 The presence of transcripts encoding MuSK isoforms with distinct extracellular domains in dev

181 ved for the two nitro musks (musk xylene and musk ketone) are significantly lower ranging between MDL

182 How Agrin binds Lrp4 and stimulates MuSK kinase activity is poorly understood.

183 with MuSK, binds neural agrin and stimulates MuSK kinase activity.

184 ciation between Lrp4 and MuSK and stimulates MuSK kinase activity.

185 recruited to MuSK, Dok-7 directly stimulates MuSK kinase activity.

186 Finally, we show that MuSK knockout mice display similar neural crest cell mig

187 of MuSK by PDZRN3 plays an important role in MuSK-mediated nicotinic acetylcholine receptor clusterin

188 nteraction with the receptor tyrosine kinase MuSK, mediates accumulation of acetylcholine receptors (

189 Formation of the Dok7/MuSK/membrane complex is required for the activation of

190 vide insight into the unique pathogenesis of MuSK MG and provide clues toward development of specific

191 in mice have shown that IgG4 antibodies from MuSK MG patients cause disease without requiring complem

192 We studied 12 MuSK-MG patients and recruited 14 AChR-MG patients match

193 areas with T1W high signal were increased in MuSK-MG patients and the intensity of the signal on axia

194 ) and orbicularis oculi (O.oculi) muscles in MuSK-MG patients compared with healthy controls, whereas

195 AChR-MG and MuSK-MG subjects displayed distinct gene segment usage b

196 signal on axial T1W sequences was greater in MuSK-MG than in controls.

197 The VL repertoire of MuSK-MG was specifically characterized by reduced V-J se

198 MG with MuSK antibodies (MuSK-MG) is often associated with persistent bulbar invo

199 eceptor (AChR-MG) or muscle specific kinase (MuSK-MG).

200 K; MuSK antibody positive myasthenia gravis (MuSK-MG)] make up a variable proportion of the remaining

201 sequencing of the BCR repertoire of AChR-MG, MuSK-MG, and healthy subjects to generate approximately

202 The extent of muscle wasting in MuSK-MG, and whether it is also found in the few acetylc

203 normalities were unique to either AChR-MG or MuSK-MG, indicating that the repertoires reflect the dis

204 cant muscle atrophy and fatty replacement in MuSK-MG, which was not found in the AChR-MG patients.

205 phosphorylates the receptor tyrosine kinase MuSK (muscle specific receptor tyrosine kinase) at the n

206 cle depends on the receptor tyrosine kinase, MuSK (muscle, skeletal receptor tyrosine-protein kinase)

207 4) to activate the receptor tyrosine kinase MuSK (muscle-specific kinase).

208 tamination levels observed for the two nitro musks (musk xylene and musk ketone) are significantly lo

209 Antibodies to muscle specific kinase [MuSK; MuSK antibody positive myasthenia gravis (MuSK-MG)

210 amount of colocalization between coexpressed MuSK mutants and chimeras confirm these results.

211 ational analysis, using coexpressed pairs of MuSK mutants and chimeras, demonstrates that the putativ

212 Biochemical analyses of MuSK mutants introduced into MuSK(-/-) myotubes demonstr

213 rm of CMS and indicate that the inability of MuSK mutants to interact with Dok-7, but not with Lrp4 o

214 The identified MUSK mutations M605I and A727V are both located in the k

215 ant of the pathogenesis of the CMS caused by MUSK mutations.

216 th more frequent respiratory crises than non-MuSK myasthenia gravis.

217 cal analyses of MuSK mutants introduced into MuSK(-/-) myotubes demonstrate that residues in this hyd

218 tin biosensor we show that in the absence of MuSK neural crest cells fail to retract non-productive l

219 yclic musk odorants or a macrocyclic diester musk odorant.

220 0 responds to macrocyclic ketone and lactone musk odorants but not to polycyclic musk odorants or a m

221 lactone musk odorants but not to polycyclic musk odorants or a macrocyclic diester musk odorant.

222 pable of activating complement when bound to MuSK on the cell surface.

223 ion of a functional complex between Lrp4 and MuSK on the surface of myotubes in the absence of the tr

224 not form in their absence, and mutations in MuSK or downstream effectors are a major cause of a grou

225 d locus, unplugged FL, encodes the zebrafish MuSK ortholog.

226 This follows MuSK partition into lipid rafts and requires its activat

227 o coordinate the cross-talk between the LRP4-MuSK pathway and integrin-focal adhesion pathway.

228 identification of a new player in the agrin-MuSK pathway, Tid1, which directly interacts with MuSK a

229 lated protein 4-muscle-specific kinase (LRP4-MuSK) pathway.

230 nvolved in GABAergic, cholinergic, and Agrin-MuSK pathways.

231 terocyclic amines, antioxidants, UV filters, musks, PBDEs and parabens.

232 Polycyclic musks (PCMs) are synthetic fragrance compounds used in p

233 aromatic hydrocarbons (PAHs), and polycyclic musks (PCMs) were correlated with sources at a scale of

234 aromatic hydrocarbons (PAHs) and polycyclic musks (PCMs).

235 yotubes, the initial stages of agrin-induced MuSK phosphorylation and AChR clustering are normal, but

236 caused severe impairment of agrin-dependent MuSK phosphorylation, aggregation of acetylcholine recep

237 only moderate impairment of agrin-dependent MuSK phosphorylation, aggregation of AChRs and interacti

238 motor neuron-derived ligand that stimulates MuSK phosphorylation, play critical roles in synaptic di

239 rin, which binds Lrp4 and stimulates further MuSK phosphorylation, stabilizing nascent synapses.

240 MuSK and Lrp4, and inhibit Agrin-stimulated MuSK phosphorylation.

241 The receptor tyrosine kinase MuSK plays a crucial role-both as a signaling molecule a

242 We conclude that MuSK plays an important role in brain functions, includi

243 tural epitope in the first Ig-like domain of MuSK, prevent binding between MuSK and Lrp4, and inhibit

244 wever, clustering of muscle specific kinase (MuSK) proceeded normally in the gamma-null muscles.

245 B mutants unable to bind to DNA also inhibit MuSK promoter activity, suggesting a CRE-independent inh

246 Mutation of this element increases the MuSK promoter activity, suggesting a role for CREB1 in a

247 xpression by small interfering RNA increases MuSK promoter activity.

248 The N-terminus of the MuSK protein, however, is sufficient to recruit another

249 pic interactions to mediate co-clustering of MuSK, rapsyn, and acetylcholine receptors at the NMJ.

250 Although agrin-MuSK-rapsyn signaling is essential for the focal innerva

251 lized by motor neurons to stimulate the LRP4-MuSK receptor in muscles for neuromuscular junction (NMJ

252 In zebrafish, the MuSK receptor initiates neuromuscular synapse formation

253 propose that Wnt-induced trafficking of the MuSK receptor to endosomes initiates a signaling cascade

254 membrane protein that is associated with the MuSK receptor tyrosine kinase.

255 Phosphorylated MuSK recruits docking protein-7 (Dok-7), an adaptor prot

256 ever, how signal is transduced from agrin to MuSK remains unclear.

257 o identified plasma membrane subdomains, and MuSK's association with itself is specific, as MuSK clus

258 substrate of MuSK, but also an activator of MuSK's kinase activity.

259 f the transmembrane domain, does not abolish MuSK self-association.

260 The homologous structure in the Asian musk shrew (Suncus murinus) is a single cluster in the l

261 xons within the vagus nerve of a mammal, the musk shrew Suncus murinus.

262 vernosus and ischiocavernosus muscles of the musk shrew.

263 Male musk shrews also have significantly larger soma areas in

264 a coreceptor of agrin that is necessary for MuSK signaling and AChR clustering and identify a potent

265 te AChR clustering by facilitating the agrin/MuSK signaling and the interaction between the receptor

266 ults provide new insight into the agrin-LRP4-MuSK signaling cascade and NMJ formation and represent a

267 ntially novel mechanism that regulates agrin/MuSK signaling cascade.

268 Its expression enables agrin binding and MuSK signaling in cells that otherwise do not respond to

269 propose that Wnt ligands activate unplugged/MuSK signaling in muscle fibers to restrict growth cone

270 ent of neuromuscular disorders linked to Wnt-MuSK signaling pathway deficiency.

271 chanism involving Hedgehog/Gli and unplugged/MuSK signaling pathways.

272 aptic differentiation are dependent on Agrin/MuSK signaling without a requirement for a secondary sig

273 biquitin ligase as an important regulator of MuSK signaling.

274 o a lesser extent for bisphenol A, synthetic musks, some pesticides, and PAHs.

275 ral lines of evidence suggest that agrin and MuSK stimulate synapse-specific transcription indirectly

276 Lrp4 can bind and stimulate MuSK, strongly suggesting that association between Lrp4

277 that biglycan is an extracellular ligand for MuSK that is important for synapse stability.

278 of muscle-specific receptor tyrosine kinase (MuSK), the key organizer of postsynaptic development at

279 ndent of the muscle-specific tyrosine kinase MuSK, the known binding partner of Dok-7 at the NMJ.

280 rine pesticides, PBDE-10, PBDE-28, PBDE-116, musk tibetene, and pentachloronitrobenzene.

281 Like MuSK, Tid1 colocalizes with AChRs at developing, adult,

282 osin Receptor Kinase (TrKA), is required for MuSK to bind Lrp4.

283 stribution does not result from targeting of MuSK to identified plasma membrane subdomains, and MuSK'

284 synaptic acetyl-choline receptor (nAChR) and MUSK transcription whereas forced expression of HDAC4 mi

285 In agreement, CREB1 could inhibit a mutant MuSK transgene reporter whose CRE site was mutated.

286 Using inducible unplugged/MuSK transgenes, we show that organization of the centra

287 show that in vivo, wnt11r and wnt4a initiate MuSK translocation from muscle membranes to recycling en

288 nockdown of several core components disrupts MuSK translocation to endosomes, AChR localization and a

289 ells attenuates agrin binding, agrin-induced MuSK tyrosine phosphorylation, and AChR clustering.

290 e level of agrin and muscle-specific kinase (MuSK) was assessed at denervated endplates.

291 Agrin and MuSK were preserved in endplates from denervated MMP3 nu

292 suggest that Lrp4 is a cis-acting ligand for MuSK, whereas Agrin functions as an allosteric and parac

293 ential for activation of the receptor kinase MuSK, which governs NMJ formation, and DOK7 mutations un

294 o visualize the cell-surface distribution of MuSK, which is found in discrete, punctate clusters.

295 demonstrate that the first Ig-like domain in MuSK, which shares homology with the NGF-binding region

296 choline receptors (AChRs) and interaction of MuSK with Dok-7, an essential intracellular binding prot

297 ion, aggregation of AChRs and interaction of MuSK with Dok-7.

298 NSF interacts directly with MuSK with nanomolar affinity, and treatment of muscle ce

299 ion levels observed for the two nitro musks (musk xylene and musk ketone) are significantly lower ran

300 quantified using one of the test chemicals, musk xylene, as a benchmark.