mdx mouse
How much dystrophin is enough: the physiological consequences of different levels of dystrophin in the mdx mouse
... Although previous studies have highlighted the pathological and functional benefits of low dystrophin levels in transgenic mice models and patient cohorts, these approaches addressed dystrophin levels required to prevent ...
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Early manifestation of alteration in cardiac function in dystrophin deficient mdx mouse using 3D CMR tagging
... in mdx mice [39]. While the observed biphasic change in mdx mice suggests that tissue tagging is more sensitive for detecting func- tional alterations than global clinical indexes, functional assessment ...
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Preventing phosphorylation of dystroglycan ameliorates the dystrophic phenotype in mdx mouse
... This study demonstrates that preventing phosphorylation of a key tyrosine residue on murine dystroglycan -Y890, ameliorates many of the main pathological symptoms associated with dystrophin deficiency in the mdx ...
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Preventing phosphorylation of dystroglycan ameliorates the dystrophic phenotype in mdx mouse
... the mdx phenotype? From the phenotypes of epidermolysis bullosa simplex with muscular dystrophy, we know that mutations in plectin contribute to sarcolemmal integrity (35-37), and that plectin is enriched at the ...
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Increased plasma lipid levels exacerbate muscle pathology in the mdx mouse model of Duchenne muscular dystrophy
... The mdx mouse is notorious for failing to exhibit the se- verity of muscle damage observed in DMD patients, dis- playing only increased centralized nuclei, little fibrosis, early myofiber necrosis ...
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Whole-body clearing, staining and screening of calcium deposits in the mdx mouse model of Duchenne muscular dystrophy
... of mdx DMD ...two mouse bodies simultaneously (mdx and ...in mdx but not control mouse ...within mdx mouse skeletal muscles of the forelimb, diaphragm, lumbar region, ...
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IL 6 signaling blockade increases inflammation but does not affect muscle function in the mdx mouse
... the mdx mouse IL-6 muscle levels are dramatically elevated, while in age matched wild-type IL-6 is negligible; indeed, it’s barely detectable via western blot in wild-type mice ...to mdx mice, but ...
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Applications of metabolomics and proteomics to the mdx mouse model of Duchenne muscular dystrophy: lessons from downstream of the transcriptome
... and mdx groups documented in ex vivo hearts perfused in the working mode contrasted with the small changes documented in these hearts freeze- clamped in vivo [54], which also agreed with recent metabolomic data ...
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The influence of sex hormones on cardiac and skeletal muscle function in the MDX mouse model of Duchenne Muscular Dystrophy
... the mdx mouse model of DMD (Anderson, McIntosh and Poettcker 1996) and Burrow (1991) found no difference in dystrophin expression when the number of somatic dystrophin reverent fibres were compared from ...
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Muscular dystrophy in the mdx mouse is a severe myopathy compounded by hypotrophy, hypertrophy and hyperplasia
... of mdx EDL muscle fibres behind that of WT during the early post- natal growth ...isolated mdx fibres during this period are attributable to the low numbers of satel- lite cells, since this is revealed as ...
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The Effect of Vitamin D Supplementation on Skeletal Muscle in the mdx Mouse Model of Duchenne Muscular Dystrophy
... Despite no direct impact of VitD on muscle function in the mdx mouse, it appeared to have improved force production in the soleus and fatigue resistance in the EDL in normal muscles. The ergogenic potential ...
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Rapamycin Ameliorates Dystrophic Phenotype in mdx Mouse Skeletal Muscle
... the mdx mouse would diminish dystrophic pathology of the diseased muscle tissue by providing a more favorable balance between effec- tor T cells and Treg ...
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Temporal changes in magnetic resonance imaging in the mdx mouse
... the mdx mice and patients with DMD differs substantially, the validity of the mdx mouse as a model of DMD has been questioned and some have advocated for other models and/or double mutants ...the ...
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The Female mdx Mouse: An Unexpected Vascular Story
... female mdx mouse: increased density of vascular sections in histology, modification of the post-ischemic hyperemia profile, increase in mitochondrial oxidative rephosphorylation capacity, in striking ...
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Identification of qPCR reference genes suitable for normalizing gene expression in the mdx mouse model of Duchenne muscular dystrophy
... numbers mouse models potentially offer, and thus endeavoured to prepare a broad and comprehensive sample set of healthy and mdx mouse muscle ...the mdx mouse to show pronounced fibrotic ...
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Mitochondrial content is preserved throughout disease progression in the mdx mouse model of Duchenne muscular dystrophy, regardless of taurine supplementation
... taurine synthesis improves skeletal muscle function in the mdx mouse model for Duchenne muscular 587. dystrophy[r] ...
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Metabolic remodeling agents show beneficial effects in the dystrophin- deficient mdx mouse model
... Results: We found a gain in body and muscle weight in all treated mice. Histologic examination showed a decrease in muscle inflammation and in the number of fibers with central nuclei and an increase in fibers with ...
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The influence of sex hormones on cardiac and skeletal muscle function in the MDX mouse model of Duchenne Muscular Dystrophy
... GLOSSARY OF ABBREVIATIONS AAV adeno-associated virus ACE angiotensin converting enzyme AO antisense oligonucleotide AR androgen receptor BW body weight C57 C57BL/10ScSn mouse Ca 2+ calci[r] ...
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Preclinical Studies in the mdx Mouse Model of Duchenne Muscular Dystrophy with the Histone Deacetylase Inhibitor Givinostat
... of mdx mice killed at the end of the treatment with different doses of givinostat, com- pared with control vehicle-treated mdx ...from mdx mice ex- posed to 5 and 10 mg/kg/d doses of givi- nostat ...
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Altered social behavior and ultrasonic communication in the dystrophin-deficient mdx mouse model of Duchenne muscular dystrophy
... by mdx residents towards WT male intruders. In these situations mdx calls had shorter dura- tions and amplitudes, two parameters remarked as pros- odic elements conveying critical emotional or motivational ...
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