ライフサイエンスコーパス: mdx mice

1 d alleviation of the dystrophic phenotype in mdx mice.

2 gnificantly altered (P < 0.001, q < 0.01) in mdx mice.

3 old) and old (~14-months old) wild type and mdx mice.

4 he muscles of periodate-oxidized ATP-treated mdx mice.

5 tage of regenerating fibers and fibrosis) in mdx mice.

6 mpensatory mechanism for the loss of nNOS in mdx mice.

7 muscle cell progenitors expressing Pax 7 in mdx mice.

8 the phrenic and hypoglossal (XII) nerves of mdx mice.

9 ible to contraction-induced muscle damage in mdx mice.

10 pathology, inflammation, and dysfunction in mdx mice.

11 in muscles of periodate-oxidized ATP-treated mdx mice.

12 irs both autophagy and lysosome formation in mdx mice.

13 gy and Akt signaling in dystrophic muscle of mdx mice.

14 ear factor-kappa B (NF-kappaB) in 7-week-old mdx mice.

15 he skeletal and cardiac disease phenotype in mdx mice.

16 generally more severely affected than dy(3K)/mdx mice.

17 iferation and myofiber regeneration in young mdx mice.

18 teomes of wild-type and dystrophin-deficient mdx mice.

19 injury and increases fibrosis in 9-month-old mdx mice.

20 n inflammation in the fore- and hindlimbs of mdx mice.

21 tent or oxidative phosphorylation defects in mdx mice.

22 avates nor alleviates cardiomyopathy in aged mdx mice.

23 by activity (ie, voluntary wheel running) in mdx mice.

24 rdiomyopathy matching that of non-transgenic mdx mice.

25 from decreased mitochondrial dysfunction in mdx mice.

26 irror the progression of muscle pathology in mdx mice.

27 ogenic gene expression compared with control mdx mice.

28 rcise muscle damage, hypoxia, and fatigue in mdx mice.

29 s some components of dystrophic pathology in mdx mice.

30 ceptibility to contraction-induced injury in mdx mice.

31 rin and negligible additional improvement in mdx mice.

32 ly compensates for the loss of dystrophin in mdx mice.

33 e morpholino targeting exon 23 in dystrophic mdx mice.

34 ed eccentric contractions when compared with mdx mice.

35 nd myofiber hypertrophy in treated mucles in mdx mice.

36 follow disease progression in the hearts of mdx mice.

37 conformation less prone to transcription in mdx mice.

38 cue by dystrophin and utrophin constructs in mdx mice.

39 d to primary muscle cells from wild type and mdx mice.

40 ed improved heart function in Mmp9-deficient mdx mice.

41 s of MMP-9 in cardiac and skeletal muscle of mdx mice.

42 ion of ERK1/2 and Akt kinase in the heart of mdx mice.

43 ventricle dilation, and fibrosis in 1-y-old mdx mice.

44 exacerbates myopathy in dystrophin-deficient mdx mice.

45 evels of MMP-9 are increased in the heart of mdx mice.

46 pression of MMP-3 and MMP-12 in the heart of mdx mice.

47 show more severe muscle phenotypes than the mdx mice.

48 ases and activator protein-1 in myofibers of mdx mice.

49 ically distinct model of muscular dystrophy, mdx mice.

50 nic myosin heavy chain in skeletal muscle of mdx mice.

51 flow and force production, compared with the mdx mice.

52 e-related cardiac dysfunction present in the mdx mice.

53 are down-regulated, in dystrophic muscle of mdx mice.

54 ta-dystroglycan and neuronal nitric oxide in mdx mice.

55 ugmented the skeletal muscle regeneration in mdx mice.

56 enesis and enhanced myofiber regeneration in mdx mice.

57 ion into muscle fibers in muscular dystrophy mdx mice.

58 l dystrophin protein in dystrophin-deficient mdx mice.

59 nt within the dystrophic skeletal muscles of MDX mice.

60 ent systemic dystrophin splice correction in mdx mice.

61 shift towards glucose utilization in Cmah-/-;mdx mice.

62 ion of delta-Dko mice was worse than that of mdx mice.

63 ficantly improved hind limb grip strength in mdx mice.

64 nction and increased exercise performance in mdx mice.

65 nsforming growth factor-beta in myofibers of mdx mice.

66 expression of MMP-9 in dystrophic muscle of mdx mice.

67 drastically increased in skeletal muscle of mdx mice.

68 tical role in ameliorating muscle disease in mdx mice.

69 tein causes myopathy in dystrophin-deficient mdx mice.

70 on also contributes to the mild phenotype in mdx mice.

71 greatly reduced by null mutation of MBP-1 in mdx mice.

72 s muscular dystrophy in dystrophin-deficient mdx mice.

73 ant increase in DMD transcript expression in mdx mice.

74 increased in vivo glycolytic flux in Cmah-/-;mdx mice.

75 ssociated virus 9 carrying the TIPE2 gene in mdx mice.

76 re severe dystrophic pathophysiology than in mdx mice.

77 eased muscle damage when compared to regular mdx mice.

78 (10%) was found after Alk4 AON treatment in mdx mice.

79 grip strength by 60-80% over vehicle-treated mdx mice.

80 ronic injury-induced dystrophic phenotype in mdx mice.

81 in the heart and increased cardiac damage in mdx mice.

82 ctivity in Duchenne muscular dystrophy (DMD) mdx mice.

83 rovements in muscle strength and function in mdx mice.

84 previously underappreciated myofiber loss in mdx mice.

85 -micro-dystrophin (AAV-muDys) to young adult mdx mice.

86 expressed in muscles of dystrophin-deficient mdx mice.

87 disease phenotype is more severe than in B10-mdx mice.

88 imilar between control-fed and quercetin-fed mdx mice.

89 mal nNOSmu on muscle contractile function in mdx mice.

90 stores under energy-deficient conditions in mdx mice.

91 ressing green fluorescent protein (GFP) with mdx mice.

92 at their pathology is different from the B10-mdx mice.

93 e-dependent changes of diaphragm function in mdx mice.

94 ed cardiac function, relative to age-matched mdx mice.

95 and enhanced muscle function in dystrophic (mdx) mice.

96 ic muscle phenotype in dystrophin deficient (mdx) mice.

97 not in others such as dystrophin-deficient (mdx) mice.

98 anterior muscle of wild-type and dystrophic (mdx) mice.

99 tion and cause muscle fatigue in dystrophic (mdx) mice.

100 pproximately 50% green cells in the blood of mdx mice) 2-weeks after parabiotic pairing.

101 Treatment of mdx mice (a DMD model) with R16/17-containing synthetic

102 Studies performed in young adult mdx mice (a mild DMD mouse model) have yielded opposing

103 In one-year-old mdx mice (a model for Duchenne muscular dystrophy, DMD),

104 d prevents functional ischemia in transgenic mdx mice, a DMD model.

105 tory and antifibrotic effects of imatinib in mdx mice, a DMD mouse model.

106 phin gene (Dmd) mutation in the germ line of mdx mice, a model for DMD, and then monitored muscle str

107 ration and function of dystrophic muscles in mdx mice, a model for Duchenne muscular dystrophy.

108 N in Duchenne muscular dystrophy (DMD) using mdx mice, a model of DMD, and by generating transgenic m

109 immunological milieu of dystrophic muscle in mdx mice, a model of DMD, to identify potential therapeu

110 contributes to the increased fatigability of mdx mice, a model of DMD.

111 tion reduces aberrant Ca(2+) influx in young mdx mice, a model of DMD.

112 lowing acute muscle injury or in muscle from mdx mice, a model of DMD.

113 deliver gene-editing components to postnatal mdx mice, a model of DMD.

114 grafted into muscles of dystrophin-deficient mdx mice, a model of Duchenne muscular dystrophy (DMD).

115 lling and necrotic disease manifestations in mdx mice, a model of Duchenne muscular dystrophy, and in

116 pproaches that rescue defective autophagy in mdx mice, a model of Duchenne muscular dystrophy, with t

117 injury have not been extensively studied in mdx mice, a murine model of Duchenne muscular dystrophy

118 ensor digitorum longus muscles in dystrophic mdx mice, a murine model of Duchenne muscular dystrophy.

119 livered to the heart of ~14-month-old female mdx mice, a phenotypic model of Duchenne cardiomyopathy.

120 dampened the local inflammatory response in mdx mice, a spontaneous mouse model of dystrophin defici

121 has not been described in DMD patients or in mdx mice, a widely used mouse model for studying DMD.

122 We assayed whether ablation of IL-10 in mdx mice affected satellite cell numbers, using Pax7 exp

123 und that delaying exogenous Akt treatment of mdx mice after the onset of peak pathology (>6 weeks) si

124 Exercised mdx mice also showed a dose-dependent increase in serum

125 Genetic ablation of the iNOS gene in mdx mice also significantly reduces muscle membrane lysi

126 dystrophy, the Y890F mice were crossed with mdx mice an established model of muscular dystrophy.

127 between muscular dystrophy and vasculature, mdx mice, an animal model for DMD, were crossed with Flt

128 se oligonucleotide-mediated exon-skipping in mdx mice and (2) stable restoration of alpha-sarcoglycan

129 iaphragm by 28% (P < 0.05) after 10 weeks in mdx mice and by 22% (P < 0.02) after 14 weeks in dko mic

130 and function were made in the same group of mdx mice and controls (housed in a non-SPF facility) usi

131 mice but unchanged in TA muscles of treated mdx mice and diaphragm of treated mdx and dko mice.

132 In mdx mice and Duchenne muscular dystrophy patients, dystr

133 Dystrophin-deficient mdx mice and dystrophin/utrophin double-knockout (dKO) m

134 c, nNOS transgene increases the endurance of mdx mice and enhances glycogen metabolism during treadmi

135 hat SSPN overexpression is well tolerated in mdx mice and improves sarcolemma defects that underlie s

136 enuates the muscular dystrophic phenotype in mdx mice and may be a potential therapeutic target in mu

137 N was substantially elevated in the serum of mdx mice and muscle biopsies after disease onset.

138 ome inhibition ameliorated cardiomyopathy in mdx mice and reduced the development of cardiac fibrosis

139 ction would decrease calcium influx in adult mdx mice and that MEMRI would be able to monitor and dif

140 erformance deficits, and gait anomalies than mdx mice and that these deficits began at a younger age.

141 gammadelta T cells to the cardiac muscle of mdx mice and to characterize their phenotype and functio

142 Muscles of both mdx mice and very old rats showed major reductions in th

143 In contrast, for muscles of mdx mice and very old rats, forces transmitted laterally

144 MD and control hiPSC-derived cardiomyocytes, mdx mice, and control mice (in the presence or absence o

145 old) and old (~14-months old) wild type and mdx mice, and human Abductor Hallucis (AH) and gastrocne

146 ed in the skeletal muscle of dKO mice versus mdx mice, and RhoA activation specifically occurred at t

147 evated in muscles from dystrophin-deficient (mdx) mice, and mdx/Stra13-/- double mutants exhibit an e

148 contrast to human patients, dystrophin-null mdx mice are only mildly affected.

149 sion levels of TIPE2 in skeletal muscle from mdx mice are significantly lower than wild-type (WT) mic

150 Akt also improves muscle function in mdx mice as demonstrated through in vivo grip strength t

151 obutamine stress was identified in the RV of mdx mice as early as 1 month.

152 identify cardiac abnormalities in the RV of mdx mice as young as 1 month, and detected myocardial fi

153 ted fibrogenesis and muscle deterioration in mdx mice, as well as exacerbated dystrophy in young PAI-

154 mdx mice at 4-5 months of age were subjected to two diff

155 so restored dystrophin protein expression in mdx mice at 6 wk after cell treatment that was further i

156 to distinguish between the WT wild type and mdx mice at any time point.

157 ator of cellular metabolism and survival, in mdx mice at pre-necrotic (<3.5 weeks) ages and demonstra

158 M2 phenotype and improved motor function of mdx mice at that later stage of the disease.

159 e in vivo or improve gross motor function of mdx mice at the early, acute peak of pathology.

160 nistration, and was 26% greater than control mdx mice at this time.

161 In 6-week-old mdx/mdx mice, blood leukocytes, including T cells, were CD62

162 t in the quadriceps muscles of 4-wk-old male mdx mice but no profound differences were observed in th

163 trophic symptoms in the limb muscle of young mdx mice, but did not prevent degeneration and regenerat

164 the diaphragm of mdx((5)cv) mice compared to mdx mice, but similar force generation in the extensor d

165 olume with subsequent reduced SV compared to mdx mice by 24 weeks.

166 cine increased voluntary running distance in mdx mice by 90% (P < 0.05) after 2 weeks and by 60% (P <

167 We also show that eosinophil depletions of mdx mice by injections of anti-chemokine receptor-3 redu

168 phosphate-sialic acid hydroxylase)-deficient mdx mice (Cmah-/-;mdx) have an accelerated cardiac pheno

169 In young mdx mice, combined dystrophin and myostatin exon skippin

170 ls of the repressive histone mark H3K9me3 in mdx mice compared to wild-type mice, indicating a chroma

171 sed fibrosis in skeletal muscle fibers of D2-mdx mice compared with B10-mdx and control.

172 traction was increased in batimastat-treated mdx mice compared with those treated with vehicle alone.

173 ac contractility and caused 95% mortality in mdx mice, contractility was preserved with only 19% mort

174 as exacerbated dystrophy in young PAI-1(-/-) mdx mice, could be reversed by miR-21 or uPA-selective i

175 we quantified muscular dystrophy measures in mdx mice deleted for Galgt2 (Galgt2(-/-)mdx).

176 Compared with WT mice, motor end-plates of mdx mice demonstrated less continuous morphology, more d

177 In mdx mice, diaphragm amplitude decreased with age and val

178 (dKO) mice are mouse models of DMD; however, mdx mice display a strong muscle regeneration capacity,

179 Cmah-/-;mdx mice display earlier functional deterioration, speci

180 ion, the dystrophin-null heart of transgenic mdx mice displayed severe cardiomyopathy matching that o

181 , in comparison to young-adult (3-month-old) mdx mice displaying only mild muscle lesions with no fib

182 ime, our study focused on old (12-month-old) mdx mice, displaying marked chronic muscle lesions, simi

183 ost normal in young-adult in contrast to old mdx mice, displaying marked microvessel alterations, and

184 wn-regulated (50-85%) in skeletal muscles of mdx mice (DMD model) vs. wild-type mice.

185 Since mdx mice do not develop dystrophic cardiomyopathy until

186 but these cells were found in the hearts of mdx mice during the study period, reaching a peak in 12-

187 When applied to muscles of mdx mice, eccentric contraction produced an acute 27% re

188 of dystrophin deficiency on nNOSbeta and use mdx mice engineered to lack nNOSmu and nNOSbeta to disce

189 In mdx mice, enhanced expression of either MuSK or rapsyn a

190 Fibers derived from ES cells of mdx mice exhibit an abnormal branched phenotype resembli

191 ted a 36% loss in torque about the ankle but mdx mice exhibited a greater torque loss of 73% (P < 0.0

192 While vehicle-treated castrated mdx mice exhibited cardiopulmonary impairment and fibros

193 In contrast, TA muscles from gsg(-/-) and mdx mice exhibited heightened P-ERK1/2 and increased nuc

194 We show that transgenic mdx mice expressing a full-length dystrophin/utrophin ch

195 ons to chronic myotendinous strain injury in mdx mice expressing a microdystrophin transgene (micrody

196 elevated to that of mdx levels in transgenic mdx mice expressing nearly full-length dystrophin.

197 to downhill treadmill running, wild-type and mdx mice expressing recombinant dystrophin in skeletal m

198 Treatment of mdx mice for 4 weeks with an established AMPK agonist, A

199 gh characterization of myofiber pathology in mdx mice from 2 weeks to 2 years of age.

200 d Cx43 function prior to challenge protected mdx mice from arrhythmogenesis and death, while mdx:utr

201 Myocytes from mdx mice had a higher incidence of isoproterenol-induced

202 The 9-month-old mdx mice had elevated Penh values and decreased breathin

203 Galgt2(-/-) mdx mice had increased heart and skeletal muscle patholo

204 As expected, transgenic mdx mice had minimal skeletal muscle disease and they al

205 However, unlike patients with DMD, mdx mice have a very mild motor function deficit, posing

206 n AAV-uUtro to neonatal dystrophin-deficient mdx mice, histological and biochemical markers of myonec

207 s or their cell extracts into the muscles of mdx mice (i.e., a mouse model of Duchenne Muscular Dystr

208 Conversely, weekly steroid treatment in mdx mice improved muscle function and histopathology and

209 Deletion of Mmp9 gene in mdx mice improved skeletal muscle structure and function

210 reduces muscle membrane lysis in 4-week-old mdx mice in vivo.

211 Therefore, we generated knockin mdx mice in which the Cx43 serine-triplet was replaced w

212 improvement in dystrophic pathophysiology in mdx mice, in vivo.

213 Ablation of IL-10 expression in mdx mice increased muscle damage in vivo and reduced mou

214 r, over-expression of nNOS in the muscles of mdx mice increased serum NO and normalized cell prolifer

215 In mdx mice, increased m-calpain levels in dystrophic soleu

216 Diaphragm function in mdx mice is commonly evaluated by specific force measure

217 -null (Sgcd(-/-)) mice and dystrophin mutant mdx mice is dramatically improved by skeletal muscle-spe

218 iR-133b, the tibialis anterior muscle of P30 mdx mice is smaller in size and exhibits a thickened int

219 roves muscle structure and function in young mdx mice, its continued inhibition causes more severe my

220 Intriguingly, in the skeletal muscle of mdx mice lacking dystrophin, we discover that the expres

221 nerative myogenesis and dystrophin-deficient mdx mice lacking Mkp5 exhibited an attenuated dystrophic

222 We report that mdx mice lacking the RNA component of telomerase (mdx/mT

223 il >/=21 months of age, we reasoned that old mdx mice may represent a better model to assess the impa

224 In mdx mice, MKP-1 deficiency reduced body weight, muscle m

225 taking advantage of Flk1(GFP/+) crossed with mdx mice (model for human DMD where all blood vessels ex

226 In mdx mice, mSSPN overexpression improves dystrophic patho

227 oteasome dysregulation in affected hearts of mdx mice (murine animal model of DMD) and cardiomyocytes

228 Mechanical anisotropy in WT wild type and mdx mice muscle were compared by using t test and one-wa

229 In dystrophic humans and mdx mice, mutations in the dystrophin gene disrupt the s

230 disease and they also outperformed original mdx mice on treadmill running.

231 d the EMG measures decreased after injury in mdx mice only.

232 f two members of this complex, dystrophin in mdx mice or alpha sarcoglycan in Sgca(-/-) mice, results

233 a model of DMD, and by generating transgenic mdx mice overexpressing ApN.

234 portantly, the GFP + cells isolated from the mdx mice (paired with GFP mice) underwent myogenic diffe

235 ls exert protective effects on the hearts of mdx mice, possibly by selectively killing pathogenic mac

236 nNOS depletion from mdx mice prevented compensatory skeletal muscle cell hyp

237 However, ablation of MBP-1 expression in mdx mice produces other effects on muscular dystrophy.

238 vels in the serum), which in 12-week-old mdx/mdx mice reduces blood T cell competence to adhere to ca

239 ent vastly improved overall muscle health in mdx mice, reducing plasma creatine kinase activity, an e

240 isms leading to increased levels of MMP-9 in mdx mice remain unknown.

241 Therefore, Cmah-/-;mdx mice represent an appropriate model for evaluating c

242 letal muscle compared with the wild-type and mdx mice, respectively.

243 selectively in the dystrophic muscles of the mdx mice restored metabolic and angiogenic gene expressi

244 Expression of a klotho transgene in mdx mice restored their longevity, reduced muscle wastin

245 PNADMD into the tibealis anterior muscles of mdx mice resulted in approximately 3-fold higher numbers

246 erodimer and that increasing beta1D chain in mdx mice results in more functional integrin at the sarc

247 Analysis of sera from 1 week to 7 months old mdx mice revealed age-dependent changes in the level of

248 Like humans, dystrophin-deficient mice (mdx mice) show cardiac dysfunction as evidenced by a dec

249 Dag1(Y890F/Y890F)/mdx mice showed a significant improvement in several par

250 In addition, D2-mdx mice showed fewer central myonuclei and increased ca

251 After six months, exercised mdx mice showed improved tetanic and specific force comp

252 We found that D2-mdx mice showed significantly reduced skeletal muscle fu

253 In mdx mice SR8278 increased lean mass and muscle function,

254 uscle cells from patients with Becker MD and mdx mice subjected to exon skipping exhibited inhibited

255 Ca(2+) leak and attenuated cardiomyopathy in mdx mice, suggesting that enhanced PKA phosphorylation o

256 ct was not observed in dystrophin-deficient (mdx) mice, suggesting that accelerated degeneration indu

257 Compared with mdx mice that developed age-dependent heart failure, mdx

258 in nNOS transgenic muscles and muscles from mdx mice that express the nNOS transgene.

259 ildtype mice, which was in stark contrast to mdx mice that had a 55% reduction in M-wave RMS (P < 0.0

260 muscular dystrophy and dystrophin-deficient mdx mice that phenocopy this disorder.

261 ency happens between 3 and 8 weeks of age in mdx mice, the animal model of DMD.

262 In AICAR-treated mdx mice, the exaggerated sensitivity of mdx diaphragm m

263 myocardial fibrosis in 6, 9 and 12-month-old mdx mice, the extent of fibrosis correlating with the de

264 imal models of DMD: (i) dystrophin-deficient mdx mice, the most commonly utilized model of DMD, which

265 In untreated mdx mice, the usual effect of muscle contraction to atte

266 an SSPN is expressed at three-fold levels in mdx mice, this increase in adhesion complex abundance im

267 muscle-specific micro-dystrophin transgenic mdx mice to 23 months and examined the cardiac phenotype

268 administered quercetin (0.2%) in 2 month old mdx mice to improve respiratory function and end-point f

269 In mdx mice treated with a peptide-conjugated phosphorodiam

270 In mdx/mdx mice treated with Brilliant Blue G, a P2X7 blocker,

271 protective effect of MuSK-GFP in muscles of mdx mice was associated with increased immunolabelling f

272 In contrast to age-matched mdx mice, we observed that both the number and regenerat

273 Reductions in muscle integrity in nNOS-null mdx mice were accompanied by decreases in specific force

274 Diaphragm movement amplitude values for mdx mice were considerably lower than those for wild-typ

275 Mdx mice were crossed to ones with cardiac myocyte-speci

276 mdx mice were crossed with RyR2-S2808A mice, in which PK

277 To do this, mdx mice were exposed to three daily 90-minute 1.3 atmos

278 Beginning at 8 weeks of age, mdx mice were fed a standard diet supplemented with 1% s

279 etermine their therapeutic value, dystrophic mdx mice were subject to forced exercise to model the DM

280 eta signaling improves respiratory function, mdx mice were treated from 2 weeks of age to 2 months or

281 Here we show that in aged (>15-month-old) mdx mice, when the pathology is significantly more sever

282 ion compared to myoblasts from wild type and mdx mice, whereas the dko mice show histological abnorma

283 In the hearts of mature Mdx mice (which have a point mutation in Dmd)-a model of

284 emetry monitoring, the authors observed that mdx mice, which lack dystrophin, had an arrhythmic death

285 ular miRNA signature in dystrophin-deficient mdx mice, which shows profound dose-responsive restorati

286 ed by EOM SCs isolated from dystrophin-null (mdx) mice, while SCs from muscles affected by dystrophin

287 d by breeding male or female dystrophin-null mdx mice with a wild type mate.

288 d mdx mice with ApN knockout mice, to obtain mdx mice with ApN depletion.

289 in, we took an opposite approach and crossed mdx mice with ApN knockout mice, to obtain mdx mice with

290 improved muscle histology compared with the mdx mice with decreased fibrosis, calcification and memb

291 We treated mdx mice with intraperitoneal injections of imatinib at

292 We then crossed mdx mice with mice null for caspase-12, the murine equiv

293 Treatment of mdx mice with morpholino oligomers to induce exon skippi

294 in and pharmacologic treatment of dystrophic mdx mice with recombinant osteoprotegerin muscles.

295 Finally, in old mdx mice with severe muscle degeneration, simvastatin en

296 Treatment of mdx mice with Sunitinib demonstrated decreased membrane

297 Treatment of one-month old mdx mice with the most effective let-7c PMO (i.e. S56) r

298 s were dramatically reduced in Sgcd(-/-) and mdx mice with the SERCA1 transgene, which also rescued t

299 scles and cardiac tissue in adult dystrophic mdx mice, with a single low-dose injection of peptide-co

300 deficits in the cardiac performance of aged mdx mice, with no effect on normal cardiac function in W