ライフサイエンスコーパス: utrophin

1 raction with UTRN mRNA and thus upregulating utrophin.

2 roblasts of mice lacking both dystrophin and utrophin.

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

4 melting transitions identical to full-length utrophin.

5 erated mice lacking both alpha7 integrin and utrophin.

6 vert dystrophic pathology or upregulation of utrophin.

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

8 e actin binding properties of dystrophin and utrophin.

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

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

11 t mdx muscle is rescued by overexpression of utrophin.

12 ed therapy and potential new unique roles of utrophin.

13 Using a utrophin 5'3'UTR reporter assay, we performed a high-thr

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

15 Utrophin, a dystrophin ortholog that is normally localiz

16 Upregulation of utrophin, a dystrophin related protein, is considered a

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

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

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

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

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

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

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

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

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

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

27 ctin in cells and in vitro, we perturbed the utrophin actin-binding domain by making point mutations

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

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

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

31 sion of surrogate muscle proteins, including utrophin, agrin, laminins, and integrins.

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

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

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

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

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

37 the benefits of dystrophin with increases of utrophin, an autosomal paralogue of dystrophin.

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

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

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

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

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

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

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

45 ssociated with increased immunolabelling for utrophin and beta-dystroglycan in the sarcolemma.

46 Increases in sarcolemmal immunolabelling for utrophin and beta-dystroglycan suggest a mechanism for t

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

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

49 3D reconstruction of F-actin decorated with utrophin and dystrophin actin-binding constructs were pe

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

51 Our results demonstrate that utrophin and dystrophin are functionally interchangeable

52 Utrophin and dystrophin can be co-expressed and co-local

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

54 Utrophin and dystrophin link cytoskeletal F-actin filame

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

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

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

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

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

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

61 data illustrate the importance of monitoring utrophin and MyHC-emb levels in the preclinical evaluati

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

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

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

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

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

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

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

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

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

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

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

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

74 nical properties of spectrin-like repeats in utrophin are more in line with the PEVK and Ig-like repe

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

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

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

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

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

80 ntial future combinations of dystrophin- and utrophin-based strategies.

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

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

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

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

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

86 However, utrophin binds laterally along actin filaments through c

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

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

89 Dystrophin and utrophin both stabilized preformed actin filaments from

90 expression in DMD patients(6) should protect utrophin by central immunologic tolerance.

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

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

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

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

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

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

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

98 Transgenic overexpression of utrophin causes broad sarcolemma localization of utrophi

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

100 ive regulator of actin binding, we find that utrophin CH1-CH2 affinity is both increased and decrease

101 serve nonuniform subcellular localization of utrophin CH1-CH2 that depends on the N-terminal flanking

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

103 within the N-terminal actin-binding half of utrophin compared to those in the C-terminal dystroglyca

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

105 anical properties of structurally homologous utrophin constructs and suggest that utrophin may functi

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

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

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

109 stabilizing chemical surrogate in dystrophin/utrophin deficiency.

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

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

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

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

114 These findings support a model in which utrophin-derived therapies might be used to treat clinic

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

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

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

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

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

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

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

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

123 The dystrophin-/-/utrophin-/-/ double knockout (dKO-Hom) mouse is a murine

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

125 We also evaluate the correlation between utrophin, dystrophin and MyHC-emb in wild-type (wt) and

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

127 inopathy critically depends on the amount of utrophin expressed.

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

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

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

131 has potential applicability for upregulating utrophin expression as a therapeutic approach for DMD.

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

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

134 Further, utrophin expression developmentally precedes that of dys

135 However, enhanced utrophin expression did not mitigate disease.

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

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

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

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

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

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

142 Utrophin expression is repressed at the post-transcripti

143 Utrophin expression is temporally and spatially regulate

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

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

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

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

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

149 Constitutive utrophin expression, a structural and functional paralog

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

151 that SSPN regulates Akt signaling to control utrophin expression.

152 sed mitochondrial biogenesis or up-regulated utrophin expression.

153 ophin expression or complementary dystrophin/utrophin expression.

154 ortant impetus for identifying activators of utrophin expression.

155 ted using an orthogonal assay for endogenous utrophin expression.

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

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

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

159 and increased expression of the compensatory utrophin gene.

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

177 In contrast, the absence of utrophin in the dystrophin-deficient double-knockout mic

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

179 Similarly, overexpression of utrophin in the transgenic mdx-Fiona mice reduced the nu

180 er functional improvement, overexpression of utrophin in wt mice results in a significant supra-funct

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

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

183 Utrophin is a dystrophin homolog expressed at high level

184 Utrophin is a dystrophin homologue found in both muscle

185 Utrophin is a fetal homologue of dystrophin that can sub

186 Utrophin is a homolog of dystrophin, the defective prote

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

188 In dystrophic muscles, utrophin is increased as part of the repair process and

189 Utrophin is normally confined to the neuromuscular junct

190 Utrophin is normally restricted to the neuromuscular jun

191 Utrophin is required for the rhBGN therapeutic effect.

192 Utrophin is the autosomal homolog of dystrophin, the pro

193 Utrophin is the autosomal homologue of dystrophin, the p

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

195 using exon-skipping, together with increased utrophin levels restores dystrophic muscle function to w

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

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

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

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

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

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

202 ologous utrophin constructs and suggest that utrophin may function as a stiff elastic element in seri

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

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

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

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

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

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

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

210 Utrophin modulation is a promising therapeutic strategy

211 The first-in-class utrophin modulator ezutromid/SMT C1100 was developed fro

212 d]oxazole (ezutromid, 1) is a first-in-class utrophin modulator that has been evaluated in a phase 2

213 wing on from ezutromid, the first-generation utrophin modulator, we describe the development of a sec

214 g their potential utility as next-generation utrophin modulators suitable for progression toward a fu

215 be the development of a second generation of utrophin modulators, based on the bioisosteric replaceme

216 y and help development of new generations of utrophin modulators.

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

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

219 2 myoblasts in vitro, a 2-fold increase in A-utrophin mRNA level was observed.

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

221 the 5' and 3' untranslated regions (UTRs) of utrophin mRNA significantly limit the magnitude of utrop

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

223 Dystrophic mdx and dystrophin-utrophin null (dko) mice were treated with glycine or L-

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

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

226 Upregulation of endogenous utrophin offers great promise for treating DMD, as it ca

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

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

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

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

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

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

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

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

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

236 the mdx mouse that remain despite transgenic utrophin overexpression.

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

238 TS) for small molecules capable of relieving utrophin post-transcriptional repression.

239 The utrophin promoter A is transcriptionally regulated in pa

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

241 O (i.e. S56) resulted in ca. two-fold higher utrophin protein expression in skeletal muscles and the

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 ncrease of utrophin whereas higher levels of utrophin reduce wt dystrophin, suggesting a finite numbe

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

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

251 Dystrophin-related protein (or utrophin) retains most of the structural and protein bin

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 her reported AhR antagonists also upregulate utrophin, showing that this pathway, which is currently

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

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

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

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

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

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

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

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

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

267 Upregulation of utrophin to compensate for the missing dystrophin offers

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

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

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

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

272 tabilize utrophin protein without increasing utrophin transcription.

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

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

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

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

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

278 in mRNA significantly limit the magnitude of utrophin upregulation achievable by promoter activation.

279 ring hit, Trichostatin A (TSA), demonstrated utrophin upregulation and functional improvement in the

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

281 Thus, utrophin upregulation strategies may be applied to the m

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

283 ort the first mechanical characterization of utrophin using atomic force microscopy (AFM).

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

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

286 synthetic transgene encoding a miniaturized utrophin (uUtro), deliverable by adeno-associated virus

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

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

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

290 reporter assays and the C2C12 cell line for utrophin western blots, to independently evaluate the si

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

292 ificantly affected by a moderate increase of utrophin whereas higher levels of utrophin reduce wt dys

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