ライフサイエンスコーパス: MuSK

1 MuSK also caused subtle changes to the structure and fun

2 MuSK antibodies are found in a variable proportion of AC

3 MuSK antibodies define a form of myasthenia gravis that

4 MuSK antibodies per se may predispose to muscle thinning

5 MuSK antibody positive patients represent a unique subse

6 MuSK expression is tightly regulated during development,

7 MuSK gene expression is regulated by nerve-derived agrin

8 MuSK interacts with the Wnt morphogens, through its Friz

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 MuSK-GFP caused an unexpected decay in nerve-evoked teta

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

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

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

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

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

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

21 osphorylation of the AChR, without affecting MuSK activation.

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

41 ator to promote association between Lrp4 and MuSK.

42 Key genes, such as Agrin, Lrp4, and MuSK, are required for the initial formation, subsequent

43 develop antibodies against agrin, LRP4, and MuSK.

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

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

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

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

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

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

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

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

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

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

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

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

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

57 loping mammalian neuromuscular junction, but MuSK might also be protective in some neuromuscular dise

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

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

60 ncoding either MuSK or rapsyn (a cytoplasmic MuSK effector protein) fused to green fluorescent protei

61 ific kinase (MuSK) and rapsyn (a cytoplasmic MuSK effector protein) in the tibialis anterior muscle o

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

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

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

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

66 eno-associated viral vectors encoding either MuSK or rapsyn (a cytoplasmic MuSK effector protein) fus

67 In mdx mice, enhanced expression of either MuSK or rapsyn ameliorated the acute loss of muscle forc

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

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

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

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

72 Affinity of the UCA Fabs for MuSK was 100-fold lower than the subnanomolar affinity o

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 uscular junction, suggesting novel roles for MuSK in muscle physiology and pathophysiology.

80 synaptic effects, suggesting novel roles for MuSK signalling in muscle physiology and pathophysiology

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

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

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

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

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

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

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

88 that the autoantigen drives autoimmunity in MuSK MG through the accumulation of somatic mutations su

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

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

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

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

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

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

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

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

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

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

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

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

101 inity maturation may contribute to increased MuSK-binding affinity.

102 xpression by small interfering RNA increases MuSK promoter activity.

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

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

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

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

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

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

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

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

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

112 ling endosomes and that this localization is MuSK dependent.

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

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

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

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

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

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

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

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

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

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

123 te the expression of muscle specific kinase (MuSK) and rapsyn (a cytoplasmic MuSK effector protein) i

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

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

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

127 Muscle specific kinase (MuSK) has a well-defined role in stabilizing the develop

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

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

130 ine receptor (AChR), muscle-specific kinase (MuSK) or other AChR-related proteins in the postsynaptic

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

147 Pathogenic muscle-specific tyrosine kinase (MuSK)-specific IgG4 autoantibodies in autoimmune myasthe

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

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

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

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

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

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

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

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

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

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

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

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

160 novel signaling pathway by which Agrin-LRP4-MuSK induces tyrosine phosphorylation of Rapsn, which is

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

162 e that appears independent of the Agrin-LRP4-MuSK-DOK7 acetylcholine receptor clustering pathway.

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

180 ne complex is required for the activation of MuSK.

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

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

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

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

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

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

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

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

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

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

191 27V are both located in the kinase domain of MuSK.

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

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

194 est a mechanism for the protective effect of MuSK in mdx muscles.

195 The protective effect of MuSK-GFP in muscles of mdx mice was associated with incr

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

197 Elevating the expression of MuSK or rapsyn revealed several distinct synaptic and ex

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

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

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

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

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

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

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

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

206 s accompanied by tyrosine phosphorylation of MuSK and betaAChR.

207 for proper folding of Ig1 and processing of MuSK.

208 n essential intracellular binding protein of MuSK.

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

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

211 biquitin ligase as an important regulator of MuSK signaling.

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

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

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

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

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 mdx muscles overexpressing MuSK-GFP or rapsyn-GFP exhibited significantly milder fo

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

227 protein) fused to green fluorescent protein (MuSK-GFP and rapsyn-GFP, respectively).

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

245 s to neural Agrin by binding and stimulating MuSK.

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

268 In zebrafish, the MuSK receptor initiates neuromuscular synapse formation

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

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

271 of how signals are transduced from agrin to MuSK.

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

273 PDZRN3 binds to MuSK and promotes its ubiquitination.

274 CA mAbs, and mature monovalent Fabs bound to MuSK and demonstrated pathogenic capacity.

275 However, monovalent UCA Fabs bound to MuSK but did not have measurable pathogenic capacity.

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.