ライフサイエンスコーパス: utrophin

1 melting transitions identical to full-length utrophin.

2 erated mice lacking both alpha7 integrin and utrophin.

3 vert dystrophic pathology or upregulation of utrophin.

4 or more exons that led to the truncation of utrophin.

5 e actin binding properties of dystrophin and utrophin.

6 rophin was found to be more complex than for utrophin.

7 le-specific kinase (MuSK), rapsyn, erbB, and utrophin.

8 t mdx muscle is rescued by overexpression of utrophin.

9 brane proteins such as beta-dystroglycan and utrophin.

10 /utr(-/-) mice that lack both dystrophin and utrophin.

11 are also highly conserved in dystrophin and utrophin.

12 ystrophin and the dystrophin-related protein utrophin.

13 two on dystrobrevin and two on dystrophin or utrophin.

14 eral membrane targeting and association with utrophin.

15 bly through interactions with dystrophin and utrophin.

16 roblasts of mice lacking both dystrophin and utrophin.

17 was replaced with an actin-binding domain of utrophin.

18 entrated at the neuromuscular junction where utrophin, a dystrophin homologue, is expressed.

19 Utrophin, a dystrophin ortholog that is normally localiz

20 approach, we found that UTRN (which encodes utrophin, a dystrophin-related protein) at 6q24, when ex

21 tive to their genetic defect, is to modulate utrophin, a functional paralogue of dystrophin, able to

22 rsion of CRISPR/Cas9 increases the amount of utrophin, a known disease modifier in Duchenne muscular

23 months in vivo resulted in up-regulation of utrophin, a marked improvement in the mechanical propert

24 pressor factor (ERF) represses extrasynaptic utrophin-A in muscle.

25 techniques, we show that the muscle isoform utrophin-A is predominantly suppressed at the translatio

26 mediated by both the 5'- and 3'-UTRs of the utrophin-A mRNA.

27 ERF overexpression repressed utrophin-A promoter activity; conversely, small interfer

28 strated physical association of ERF with the utrophin-A promoter N-box/EBS site.

29 ulation in DMD, and they provide a model for utrophin-A regulation in muscle.

30 synapse formation and in the organization of utrophin, acetylcholine receptor, and acetylcholinestera

31 l of actin binding proposed for fimbrin, the utrophin actin-binding domain appears to associate with

32 Although there is a crystal structure of the utrophin actin-binding domain, electron microscopy of th

33 controversy concerning the structure of the utrophin-actin complex, with implications for the pathop

34 We suggest a physical model in which utrophin acts as a scaffolding protein that stabilizes l

35 ression of a "synaptic scaffold" of DAPs and utrophin along myofibers might compensate for the molecu

36 support the model for lateral association of utrophin along the actin filament and provide the molecu

37 gly, mice that lack both alpha7 integrin and utrophin (alpha7/utr(-/-)) were viable and fertile.

38 nce suggesting that spectrin-like repeats of utrophin also participate in binding to actin.

39 d several recombinant fragments encoding the utrophin amino-terminal domain alone or in combination w

40 produced from each gene: 13 and 5.5 kb from utrophin and 14 and 4.8 kb from dystrophin.

41 is essential for primary interaction between utrophin and actin, spectrin-like repeats have additive

42 entiation after CTX injury caused by loss of utrophin and Akt signaling.

43 strain injury had an increased expression of utrophin and alpha7-integrin together with the dramatic

44 MD mice is dependent on the presence of both utrophin and alpha7beta1 integrin, even when they are in

45 to laminin-binding complexes is dependent on utrophin and alpha7beta1 integrin.

46 rotubule binding activity of dystrophin with utrophin and analyzed several transgenic mouse models to

47 res ankyrin-B for localization of dystrophin/utrophin and beta-DG and for maintenance of its postnata

48 is required for association of the AChR with utrophin and beta-dystroglycan, and for the agrin-induce

49 Protein expression of utrophin and DAPs was equal to or above that of wild-typ

50 of utrophin by increasing transportation of utrophin and DG from endoplasmic reticulum/Golgi membran

51 h syntrophin, and the dystrophin homologues, utrophin and dystrobrevin, are restricted to the basolat

52 3D reconstruction of F-actin decorated with utrophin and dystrophin actin-binding constructs were pe

53 We have determined that full-length utrophin and dystrophin and the short dystrophin isoform

54 Our results demonstrate that utrophin and dystrophin are functionally interchangeable

55 ding increases in Dystrophin family members, utrophin and dystrophin Dp116, although dystroglycan rem

56 Utrophin and dystrophin link cytoskeletal F-actin filame

57 le and a subset of sympathetic neurons where utrophin and dystrophin localize at nicotinic synapses.

58 s lacking other cytoskeletal DGC components (utrophin and dystrophin) and myotubes lacking a alpha-DB

59 (CTX) injury, regenerating myofibers express utrophin and Galgt2-modified alpha-DG around the sarcole

60 muscle regeneration and the up-regulation of utrophin and integrin are thought to protect mdx muscle.

61 While both Utrophin and Lifeact robustly label F-actin structures w

62 animals (mdx/CT) increases the expression of utrophin and many DAPs, including dystroglycans, sarcogl

63 d beta-tubulin, mdx mouse hearts accumulated utrophin and MLP, and MLP-null mouse hearts accumulated

64 Without syntrophin, utrophin and neuronal nitric oxide synthase mu (nNOSmu)

65 to AChR distribution and to localization of utrophin and nNOSmu at the NMJ.

66 lso increased the surface membrane levels of utrophin and other DPC proteins, including beta-dystrogl

67 places residues that are highly conserved in utrophin and other members of the spectrin superfamily a

68 ons have undetectable levels of postsynaptic utrophin and reduced levels of acetylcholine receptor an

69 t of SSPN-null mice with viral Akt increased utrophin and restored muscle repair after injury, reveal

70 n, we have expressed full-length recombinant utrophin and show that the purified protein is fully sol

71 ated complex; by DG-dependent recruitment of utrophin and Src activation; and by integrin-dependent f

72 pair of labeling sites in the CH domains of utrophin and used dipolar electron-electron resonance to

73 s, including laminin alpha4, laminin alpha5, utrophin, and NCAM, were expressed along extrasynaptic r

74 lpha-Dystrobrevin-1 is associated with Dp71, utrophin, and syntrophin.

75 beta-DG can also associate with utrophin, and this differential association correlates w

76 d relocalization of the DAPC, dystrophin and utrophin are able to alter both structural and biochemic

77 these findings indicate that dystrophin and utrophin are critical to membrane stability-dependent ca

78 2-syntrophin and its F-actin-binding protein utrophin are enriched in subcellular fractions containin

79 Although dystrophin and utrophin are functionally homologous actin-binding prote

80 Dystrophin and utrophin are highly similar proteins that both link cort

81 and is accompanied by increased abundance of utrophin around the extra-synaptic sarcolemma.

82 phin proteins, syntrophin, dystrobrevin, and utrophin as essential GPCR-interacting proteins for alph

83 ats similar to those found in dystrophin and utrophin, as well as a domain homologous to the carboxyl

84 Our findings demonstrate that syntrophin and utrophin associate with alpha(1D)-ARs to create a functi

85 ST205 colocalized with beta 2-syntrophin and utrophin at neuromuscular junctions.

86 Systemically delivered rhBGN up-regulates utrophin at the sarcolemma and reduces muscle pathology

87 putative protein mdx biomarkers to evaluate utrophin based strategies which may help to accelerate t

88 ed with rapsyn and, to a lesser degree, with utrophin, beta-dystroglycan, MuSK, and src-related kinas

89 Dystrophin and utrophin bind actin in vitro with similar affinities, bu

90 Dystrophin and its homolog utrophin bind to cytoskeletal actin to form mechanical l

91 cation of alpha-DG glycosylation can promote utrophin binding and rescue dystrophic phenotypes in mou

92 itive to increasing ionic strength, although utrophin binding was unaffected.

93 erexpression of sarcospan, a dystrophin- and utrophin-binding protein, ameliorates mdx muscular dystr

94 However, utrophin binds laterally along actin filaments through c

95 Thus, when dystrophin or utrophin binds, actin becomes less like cast iron (stron

96 According to our data, dystrophin and utrophin both bound alongside actin filaments with submi

97 Dystrophin and utrophin both stabilized preformed actin filaments from

98 SSPN improved cell surface expression of utrophin by increasing transportation of utrophin and DG

99 hin in mice, recent studies question whether utrophin can bind laterally along actin filaments and an

100 We demonstrate that like dystrophin, utrophin can form an extensive lateral association with

101 Although the dystrophin homolog utrophin can functionally compensate for dystrophin in m

102 er in combination with a higher temperature, Utrophin can label F-actin with minimal defects.

103 xpression of the dystrophin-related protein, utrophin can prevent pathology.

104 n has similar effects, but at higher levels, utrophin caused much greater restrictions in amplitude a

105 Transgenic overexpression of utrophin causes broad sarcolemma localization of utrophi

106 The lozenge-like utrophin CH domain densities localized to the upper surf

107 mdx mice expressing a full-length dystrophin/utrophin chimera completely lacking microtubule binding

108 at ICA512 connects secretory granules to the utrophin complex and the actin cytoskeleton.

109 at the alpha7beta1 integrin, dystrophin, and utrophin complexes act in a concerted manner to maintain

110 dissociation of ICA512 from beta2-syntrophin-utrophin complexes and the cleavage of the ICA512 cytopl

111 Several miniaturized dystrophin/utrophin constructs are utilized for gene therapy, and w

112 ports of functional rescue by dystrophin and utrophin constructs in mdx mice.

113 Utrophin constructs involving N-terminal, C-terminal or

114 stabilizing chemical surrogate in dystrophin/utrophin deficiency.

115 In Duchenne muscular dystrophy myocytes (mdx/utrophin deficient), the SSC was excessive and arrhythmo

116 In dystrophin/utrophin-deficient double-knockout (dKO) mice, a more se

117 find complex, gene dose-dependent effects of utrophin depletion in dystrophin-deficient mdx muscle: (

118 Becker muscular dystrophies, and therapeutic utrophin derivatives are currently being developed.

119 stingly, swapping this microdomain back into utrophin did not convey the nNOS binding activity.

120 Depletion of utrophin disrupts domain composition in a manner that fa

121 ted by docking the crystal structures of the utrophin domain and F-actin into the reconstruction.

122 ild dystrophic phenotype and (ii) dystrophin/utrophin double knock-out (dKO) mice, which display a si

123 fect of prednisolone treatment in dystrophin/utrophin double knockout (dKO) mice, which exhibit a sev

124 nce of stem cell depletion in the dystrophin/utrophin double knockout (dKO) mouse model, which exhibi

125 While castration of dystrophin and utrophin double mutant (mdx-dm) mice to mimic pre-pubert

126 ystrophic pathology in the severe dystrophin/utrophin double mutant (mdx:utr (-/-)) mouse model of DM

127 Dystrophin-deficient mdx mice and dystrophin/utrophin double-knockout (dKO) mice are mouse models of

128 potential mdx markers specific to increased utrophin (DUS3, TPI1) and highlights novel mdx biomarker

129 e AAV-mediated exon skipping approach in the utrophin/dystrophin double-knockout (dKO) mouse which is

130 is able to ameliorate these abnormalities in utrophin/dystrophin-deficient mice.

131 rane or the abnormal oxidative properties of utrophin/dystrophin-deficient muscle.

132 inopathy critically depends on the amount of utrophin expressed.

133 We examined dystrophin and utrophin expression and localization in the avian parasy

134 Cn in skeletal muscle was shown to increase utrophin expression and reduce overall disease pathology

135 wo independently regulated promoters control utrophin expression and the upstream promoter (promoter

136 scribed here as a novel strategy to increase utrophin expression as a therapy for DMD.

137 regeneration and new pathways that regulate utrophin expression at the cell surface.

138 Further, utrophin expression developmentally precedes that of dys

139 However, enhanced utrophin expression did not mitigate disease.

140 nisms can be targeted to increase endogenous utrophin expression in cultured muscle cells.

141 recombinant human biglycan (rhBGN) increases utrophin expression in cultured myotubes.

142 racellular matrix protein biglycan regulates utrophin expression in immature muscle and that recombin

143 nding the regulatory mechanisms that control utrophin expression in muscle and may facilitate the dev

144 way offers a potential mechanism to modulate utrophin expression in muscle.

145 ministration of SMT022357 leads to increased utrophin expression in skeletal, respiratory and cardiac

146 Significantly, utrophin expression is localized along the length of the

147 Utrophin expression is temporally and spatially regulate

148 d that F-tractin, and perhaps Utrophin, when Utrophin expression levels are optimized to label effici

149 number of infiltrating macrophages, or alter utrophin expression or localization.

150 When utrophin expression was activated 30 days after birth, i

151 transgenic mouse model where muscle specific utrophin expression was conditioned by addition of tetra

152 Additionally, strong Utrophin expression within the germline causes F-actin f

153 ry mechanisms featuring miRNAs that regulate utrophin expression, and demonstrated that these mechani

154 ophin expression or complementary dystrophin/utrophin expression.

155 ortant impetus for identifying activators of utrophin expression.

156 that SSPN regulates Akt signaling to control utrophin expression.

157 sed mitochondrial biogenesis or up-regulated utrophin expression.

158 Consequently, the mdx:utrophin(-/-):Flt-1(+/-) mice display improved muscle hi

159 actin binding properties of each recombinant utrophin fragment using a high-speed sedimentation assay

160 Beauty transposon system carrying the micro-utrophin gene, differentiate these cells into skeletal m

161 and increased expression of the compensatory utrophin gene.

162 arrying mutations in both the dystrophin and utrophin genes die prematurely as a consequence of sever

163 The alpha7beta1 integrin, dystrophin, and utrophin glycoprotein complexes are the major laminin re

164 s the sarcolemma by increasing levels of the utrophin-glycoprotein complex (UGC) at the extrasynaptic

165 s) ages and demonstrated upregulation of the utrophin-glycoprotein complex and protection against con

166 -dystroglycan, but not with other dystrophin/utrophin-glycoprotein complex components, suggesting tha

167 ot cause the concomitant overexpression of a utrophin-glycoprotein complex in mdx muscles and has no

168 membrane residence of the integrins, the DGC/utrophin-glycoprotein complex of proteins and annexin A1

169 t is a component of both the dystrophin- and utrophin-glycoprotein complexes.

170 Transgenic overexpression of utrophin has been shown to significantly improve aspects

171 d pauses microtubule polymerization, whereas utrophin has no activity in either assay.

172 of saturation, the binding of dystrophin and utrophin has similar effects, but at higher levels, utro

173 ly used TPA to show that both dystrophin and utrophin have a paradoxical effect on actin rotational d

174 stribution and transcriptional regulation of utrophin have been characterized extensively, and more r

175 , to date only two different mRNA species of utrophin have been identified.

176 the structure of the actin-binding domain of utrophin in complex with F-actin, determined by cryo-ele

177 gents to effect therapeutic up-regulation of utrophin in DMD.

178 ategies to replace defective dystrophin with utrophin in individuals with muscular dystrophy requires

179 es the critical roles of alpha7 integrin and utrophin in maintaining myotendinous junction structure

180 y patients will be to increase expression of utrophin in muscle.

181 DG may also act independently of dystrophin/utrophin in non-muscle tissues.

182 Our experiments reveal functions of utrophin in regeneration and new pathways that regulate

183 ics of actin interaction with dystrophin and utrophin in relationship to the pathology of muscular dy

184 ere is no trace of this open conformation of utrophin in the absence of actin, providing strong suppo

185 Although the increase in MLP and utrophin in the mdx mouse heart was able to compensate f

186 eviously demonstrated that overexpression of utrophin in the muscles of dystrophin-null transgenic mi

187 ater expression and membrane localization of utrophin, integrins, and beta-dystroglycan, which anchor

188 r members of the spectrin superfamily at the utrophin interface with actin, confirming the likelihood

189 Utrophin is a 400 kDa autosomal homolog of dystrophin an

190 Utrophin is a chromosome 6-encoded dystrophin-related pr

191 Utrophin is a dystrophin homolog expressed at high level

192 Utrophin is a dystrophin homologue found in both muscle

193 Utrophin is a homolog of dystrophin, the defective prote

194 Utrophin is a large protein which accumulates at the neu

195 Utrophin is a large ubiquitously expressed cytoskeletal

196 ystrophy patients, dystrophin is lacking and utrophin is consequently up-regulated and redistributed

197 Utrophin is normally confined to the neuromuscular junct

198 Utrophin is normally restricted to the neuromuscular jun

199 Utrophin is required for the rhBGN therapeutic effect.

200 Utrophin is the autosomal homolog of dystrophin, the pro

201 Utrophin is the autosomal homologue of dystrophin, the p

202 Utrophin is the autosomal homologue of dystrophin.

203 lot analysis detected several putative short utrophin isoforms that may be homologs of the dystrophin

204 utionary conservation between dystrophin and utrophin isoforms, we have compared their expression pat

205 eletal muscle histopathologies in dystrophin/utrophin knockout (dys(-/-) utro(-/-) dKO) mice is close

206 We show that Akt signaling and utrophin levels were diminished in sarcospan (SSPN)-defi

207 Interestingly, utrophin levels were further increased in these mice.

208 n, possibly in conjunction with up-regulated utrophin levels, may help maintain minimal muscle force

209 vely assess the utility of three such tools--Utrophin, Lifeact, and F-tractin--for characterizing the

210 Full-length utrophin, like dystrophin, displayed a highly cooperativ

211 Utrophin, like its homologue dystrophin, forms a link be

212 rotein, focal adhesion kinase, glutaredoxin, utrophin) may be novel mediators of NFT formation or deg

213 ntegrin (mdx/alpha7(-/-)), or dystrophin and utrophin (mdx/utr(-/-)), exhibit severe muscle pathology

214 ransgene in mice lacking both dystrophin and utrophin (mdx:utrn(-/-)).

215 sates for the absence of the dystrophin- and utrophin-mediated linkage systems.

216 Up-regulation of a dystrophin homologue, utrophin, mediates selective DGC retention.

217 higher survival rates compared with the mdx:utrophin(-/-) mice, which show more severe muscle phenot

218 wild-type, mdx and Fiona (mdx overexpressing utrophin) mice.

219 cular basis for designing the most effective utrophin "mini-genes" for treatment of dystrophinopathie

220 rongly endorses the therapeutic potential of utrophin modulation as a disease modifying therapeutic s

221 asing rate, thus increasing resilience, with utrophin more effective than dystrophin.

222 te the actin binding activity of full-length utrophin more faithfully than the amino-terminal domain

223 2 myoblasts in vitro, a 2-fold increase in A-utrophin mRNA level was observed.

224 nced promoter activity as well as endogenous utrophin mRNA levels in cultured muscle cells in vitro.

225 y demonstrated that the dystrophin homologue utrophin neither binds microtubules in vitro nor rescues

226 st that MAST205 and SAST link the dystrophin/utrophin network with microtubule filaments via the synt

227 tment of young dystrophic mdx and dystrophin/utrophin null (dko) mice with BGP-15, a coinducer of hea

228 Synaptic localization of utrophin occurs in part by heregulin-mediated extracellu

229 rophin recombinant fragments and full-length utrophin on 6-propionyl-2-(N,N-dimethylamino)naphthalene

230 The effects of dystrophin and utrophin on actin dynamics provide molecular insight int

231 by determining the effects of dystrophin and utrophin on the microsecond rotational dynamics of a pho

232 e-knockout mice to test the contributions of utrophin or alpha7 integrin.

233 animal models of DMD by increasing diaphragm utrophin or dystrophin expression and thereby restoring

234 d not correlate with increased expression of utrophin or sarcoglycans, but rather caused their decrea

235 c overexpression of the dystrophin homologue utrophin, or functional dystrophin constructs in mdx mus

236 elegans genome contains a single dystrophin/utrophin orthologue, dys-1.

237 We also found that transgenic utrophin overexpression does not correct subsarcolemmal

238 the mdx mouse that remain despite transgenic utrophin overexpression.

239 ted muscles displayed large numbers of micro-utrophin-positive myofibers, with biochemically restored

240 The utrophin promoter A is transcriptionally regulated in pa

241 to the N-box/EBS and activation of the major utrophin promoter-A expressed in myofibers.

242 f miRNAs, resulting in an increased level of utrophin protein in C2C12 cells.

243 ce indicate that biglycan acts by recruiting utrophin protein to the muscle membrane.

244 acts with the UGC and functions to stabilize utrophin protein without increasing utrophin transcripti

245 Utrophin R15/16 is homologous to dystrophin R16/17.

246 crodomains with the corresponding regions of utrophin R15/16 suggests that the nNOS binding site is l

247 e actin filaments, we compared the effect of utrophin recombinant fragments and full-length utrophin

248 ect evidence, respectively, for ERF-mediated utrophin repression in vivo.

249 phin causes broad sarcolemma localization of utrophin, restoration of laminin binding and amelioratio

250 agments of dystrophin or the closely related utrophin resulted in the localization of these protein d

251 (DYSR11-17) or the homologous region of the utrophin rod domain (UTROR11-16).

252 Utrophin's ability to compensate for dystrophin during d

253 Each of utrophin's actin-binding domains promotes resilience in

254 ding constructs were performed using Utr261 (utrophin's CH domain pair), Utr416 (utrophin's CH domain

255 Utr261 (utrophin's CH domain pair), Utr416 (utrophin's CH domains and first spectrin-repeat) and Dys

256 Resolution of utrophin's CH domains and spectrin-repeats permitted doc

257 ether the autosomal homologue of dystrophin, utrophin, shared this rod domain actin binding activity.

258 er and mice deficient in both dystrophin and utrophin showed loss of the smooth muscle sarcoglycan co

259 revealing an important role for the SSPN-Akt-utrophin signaling axis in regeneration.

260 re of the second calponin homology domain of utrophin solved by X-ray crystallography, and compare it

261 reconstruction to examine the complex of the utrophin tandem CH domain with F-actin.

262 e demonstrated that the C-terminal domain of utrophin targeted to neuromuscular junctions in normal m

263 This work supports the use of a utrophin template for gene or protein therapy designs.

264 uggest an interaction between syntrophin and utrophin that leaves the PDZ domain of syntrophin availa

265 In utrophin, the amino-terminal domain and an adjacent stri

266 Similarly, utrophin, the autosomal homologue of dystrophin, is not

267 e marginal, suggesting that the age at which utrophin therapy is initiated could be an important fact

268 The new model shows that utrophin therapy, initiated after birth, can be effectiv

269 Binding of dystrophin or utrophin to actin resulted in significant changes in the

270 ts can be explained by incomplete binding of utrophin to actin, heterogeneity in the mode of binding,

271 contribution of the alpha7beta1 integrin and utrophin to muscle integrity and function, we generated

272 half of the PH1 domain were able to restore utrophin to the NMJ but did not correct the aberrant ACh

273 Sarcospan boosts levels of utrophin to therapeutic levels at the sarcolemma, where

274 ntracellular cytoskeleton (via dystrophin or utrophin) to the extracellular matrix (via laminin, agri

275 We also identified an alternatively spliced utrophin transcript that lacks the equivalent of the alt

276 suggesting that drug treatment is modulating utrophin transcription in extra-synaptic myonuclei.

277 tabilize utrophin protein without increasing utrophin transcription.

278 Similar to the effects of utrophin, transgenic overexpression of alpha7 integrin p

279 Immunostaining revealed utrophin up-regulation in both mouse strains.

280 ssors" as a potential strategy for achieving utrophin up-regulation in DMD, and they provide a model

281 sease phenotype in the mdx mouse; therefore, utrophin up-regulation is under intense investigation as

282 f dystrophic phenotype by heregulin-mediated utrophin up-regulation offers a pharmacological therapeu

283 we have identified two novel transcripts of utrophin, Up71 and Up140, with unique first exons and pr

284 ytes, and this was associated with a lack of utrophin upregulation in the dystrophic canine cardiac m

285 sociated sarcolemmal glycoproteins, increase utrophin usage, and increase laminin binding.

286 exes in skeletal muscle, the dystrophin- and utrophin (Utr)-glycoprotein complexes (DGC and UGC).

287 For example, the expression of utrophin (Utrn) is suppressed during skeletal muscle dif

288 In a transgenic mdx mouse, where utrophin was over expressed in the skeletal muscle and t

289 Interestingly, utrophin was upregulated in dystrophin-negative heart ce

290 expression of the dystrophin-related protein utrophin, we have previously demonstrated in dystrophin-

291 nculin, alpha-actinin, beta-dystroglycan and utrophin were all retained on mdx sarcolemma, indicating

292 is case, we find that F-tractin, and perhaps Utrophin, when Utrophin expression levels are optimized

293 a linkage to muscle stretches, compared with utrophin, which binds via one contiguous actin-binding d

294 s decorated with the actin-binding domain of utrophin, which contains two calponin homology domains.

295 f dystrophy promotes increased expression of utrophin, which replaces the function of dystrophin ther

296 eased expression of alpha7beta1 integrin and utrophin, which suggests that these laminin binding comp

297 ent for dystrophin and haploinsufficient for utrophin with skeletal myopathy and cardiomyopathy that

298 cal properties of full-length dystrophin and utrophin with therapeutically relevant miniaturized cons

299 actin to evaluate domains of dystrophin and utrophin, with implications for gene therapy in muscular

300 In contrast, we found that a "micro-utrophin," with more extensive internal deletions, is as