ライフサイエンスコーパス: MuSK

1 MuSK antibodies are found in a variable proportion of AC

2 MuSK antibodies define a form of myasthenia gravis that

3 MuSK antibodies per se may predispose to muscle thinning

4 MuSK antibody positive patients represent a unique subse

5 MuSK expression is tightly regulated during development,

6 MuSK gene expression is regulated by nerve-derived agrin

7 MuSK interacts with the Wnt morphogens, through its Friz

8 MuSK is a receptor tyrosine kinase essential for neuromu

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

10 MuSK is necessary for prepatterning of the endplate zone

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

12 MuSK-antibody associated MG may be different in etiologi

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

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

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

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

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

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

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

20 osphorylation of the AChR, without affecting MuSK activation.

21 The presence of antibodies against MuSK appears to define a subgroup of patients with seron

22 nd erbB receptors 2, 3, and 4 and (2) agrin, MuSK, and rapsyn and select NMJ-associated structural pr

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

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

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

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

27 hat NOS is a likely participant in the agrin-MuSK signaling pathway of skeletal muscle cells.

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

29 ce, revealing an active role of GGT in Agrin/MuSK signaling.

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

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

32 n important signaling component in the Agrin/MuSK pathway.

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

34 results from their aggregation by the agrin/MuSK signaling pathway and their synthetic up-regulation

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

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

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

38 ostsynaptic assembly downstream of agrin and MuSK.

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

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

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

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

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

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

45 ator to promote association between Lrp4 and MuSK.

46 develop antibodies against agrin, LRP4, and MuSK.

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

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

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

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

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

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

53 wo AChR antibody-positive patients, and anti-MuSK antibody levels decreased in one "AChR antibody-neg

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

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

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

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

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

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

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

61 We report here that in muscle cells, MuSK interacts with Dishevelled (Dvl), a signaling molec

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

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

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

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

66 presynaptic factor, yet the agrin-dependent MuSK signaling cascade is largely undefined.

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

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

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

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

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

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

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

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

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

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

77 Our results demonstrate a new role for MuSK homologs in axonal pathway selection.

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

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

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

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

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

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

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

85 immunoprecipitation of (125)I-labelled-human MuSK, 27 of 66 (41%) seronegative patients were positive

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

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

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

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

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

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

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

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

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

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

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

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

98 xpression by small interfering RNA increases MuSK promoter activity.

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

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

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

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

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

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

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

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

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

108 ling endosomes and that this localization is MuSK dependent.

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

110 cts by activating the muscle-specific kinase MuSK and inducing coaggregation of the 43-kDa protein ra

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

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

113 ly, it requires the receptor tyrosine kinase MuSK (muscle-specific kinase), the intracellular protein

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

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

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

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

118 activates the transmembrane tyrosine kinase MuSK to mediate acetylcholine receptor (AChR) clustering

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

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

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

122 encodes a homolog of muscle-specific kinase (MuSK) and that, unlike mammalian MuSK, unplugged has onl

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

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

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

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

127 The muscle-specific kinase (MuSK) is the key component that mediates the synapse-ind

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

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

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

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

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

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

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

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

136 aratus that includes muscle-specific kinase (MuSK), rapsyn, erbB, and utrophin.

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

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

139 antibody against the muscle-specific kinase (MuSK).

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

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

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

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

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

145 he muscle-specific receptor tyrosine kinase (MuSK) to cluster acetylcholine receptors (AChRs) on the

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

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

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

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

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

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

152 Antibodies to rat muscle specific kinase, MuSK, have recently been identified in some generalized

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

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

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

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

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

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

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

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

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

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

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

164 fic kinase (MuSK) and that, unlike mammalian MuSK, unplugged has only a limited role in neuromuscular

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

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

167 Moreover, MuSK and Abl kinases effected reciprocal tyrosine phosph

168 haracterization of the promoter of the mouse MuSK gene.

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

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

171 ellular Ig-like domains of soluble or native MuSK.

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

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

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

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

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

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

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

179 ne complex is required for the activation of MuSK.

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

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

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

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

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

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

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

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

188 in which most of the intracellular domain of MuSK is replaced by a related kinase are viable.

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

190 27V are both located in the kinase domain of MuSK.

191 racellular signaling mechanism downstream of MuSK is poorly characterized.

192 The signaling mechanism downstream of MuSK is poorly defined.

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

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

195 n-induced AChR clustering and enhancement of MuSK tyrosine phosphorylation.

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

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

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

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

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

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

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

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

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

205 s accompanied by tyrosine phosphorylation of MuSK and betaAChR.

206 for proper folding of Ig1 and processing of MuSK.

207 n essential intracellular binding protein of MuSK.

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

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

210 biquitin ligase as an important regulator of MuSK signaling.

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

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

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

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

215 The transcription of MuSK starts at multiple sites with a major site 51 nt up

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

217 eptide representing the Dok7-binding site on MuSK.

218 recent clinical and experimental studies on MuSK antibody associated myasthenia gravis, and summariz

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

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

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

222 ed uniformly in mice lacking either agrin or MuSK.

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

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

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

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

227 Rather, MuSK expression is increased in muscle cells stimulated

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

244 s to neural Agrin by binding and stimulating MuSK.

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

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

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

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

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

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

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

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

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

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

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

256 se results suggest a novel mechanism for the MuSK synapse-specific expression.

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

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

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

260 iates axonal pathfinding, independent of the MuSK downstream component rapsyn.

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

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

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

264 Interestingly, the promoter activity of the MuSK gene did not respond to neuregulin, a factor believ

265 Expression of the MuSK gene is tightly regulated during development and at

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

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

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

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

270 In zebrafish, the MuSK receptor initiates neuromuscular synapse formation

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

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

273 of how signals are transduced from agrin to MuSK.

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

275 PDZRN3 binds to MuSK and promotes its ubiquitination.

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

277 recruit another receptor tyrosine kinase to MuSK clusters.

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

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

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

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

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

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

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

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

286 ecessary and sufficient for association with MuSK.

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

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

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

290 the membrane and formation of a complex with MuSK.

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

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

293 s cause disease by directly interfering with MuSK function.

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

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

296 23.7%; P = .02), and 0 of 11 who had MG with MuSK antibodies; 0 of 29 controls had cortactin antibodi

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

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

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

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