The mdx mouse
Temporal changes in magnetic resonance imaging in the mdx mouse
... Conclusions Mdx mice have muscle pathology, but the phenotype is much less severe than that seen with DMD in ...the mdx mice and patients with DMD differs substantially, the validity of the mdx ...
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Humanizing the mdx mouse model of DMD: the long and the short of it.
... knockout mouse models described here utilize this mdx animal unless noted ...disease, mdx skeletal muscles exhibit active myo fiber necrosis, cellular infiltration, a wide range of myo fiber sizes and ...
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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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How much dystrophin is enough: the physiological consequences of different levels of dystrophin in the mdx mouse
... Abstract: Splice modulation therapy has shown great clinical promise in Duchenne muscular dystrophy, resulting in the production of dystrophin protein. Despite this, the relationship between restoring dystrophin to ...
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IL 6 signaling blockade increases inflammation but does not affect muscle function in the mdx mouse
... the mouse model (mdx) of Duchenne Muscular Dystrophy, IL-6 and muscle inflammation are elevated, which is believed to contribute to the chronic inflammation and failure of muscle regeneration in ...
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The Effect of Vitamin D Supplementation on Skeletal Muscle in the mdx Mouse Model of Duchenne Muscular Dystrophy
... 5. Conclusions In conclusion, VitD supplementation did not appear to have any beneficial effect on the contractile properties or regenerative capacity of dystrophic skeletal muscle in the mdx mouse. ...
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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 and the human disease form and Grady et ...the mdx mouse model was not a sound model of cardiomyopathy in DMD (Grady et ...the mdx mouse model in DMD research ...
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Applications of metabolomics and proteomics to the mdx mouse model of Duchenne muscular dystrophy: lessons from downstream of the transcriptome
... used, mouse model of human disease. The mdx mouse is a model of Duchenne muscular dystrophy (DMD) and has been widely used to understand the progressive skeletal muscle wasting that accompanies DMD, ...
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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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Identification of qPCR reference genes suitable for normalizing gene expression in the mdx mouse model of Duchenne muscular dystrophy
... The mdx mouse is the most widely-used animal model of the human disease Duchenne muscular dystrophy, and quantitative PCR analysis of gene expression in the muscles of this animal plays a key role in the ...
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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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Effect of Nuclear Factor κB Inhibition on Serotype 9 Adeno-Associated Viral (AAV9) Minidystrophin Gene Transfer to the mdx Mouse
... the mdx mouse model of ...C57BL/10ScSn-Dmd mdx /J (mdx) mice, the addition of octalysine (8K)–NF- κ B essential modulator (NEMO)- binding do- main (8K-NBD) peptide treatment to AAV9 ...
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Preclinical Studies in the mdx Mouse Model of Duchenne Muscular Dystrophy with the Histone Deacetylase Inhibitor Givinostat
... in mdx mice—the mouse model of Duchenne muscular dystrophy ...1.5-month-old mdx mice to optimal concentrations of givinostat promoted the formation of muscles with increased cross-sectional area and ...
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The influence of sex hormones on cardiac and skeletal muscle function in the MDX mouse model of Duchenne Muscular Dystrophy
... in mdx were not different between males and females at any age tested and that both sexes in mdx had a dampened cardiac responsiveness to exogenous ...in mdx EDL specific force generation ...
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Whole-body clearing, staining and screening of calcium deposits in the mdx mouse model of Duchenne muscular dystrophy
... microscope body, Zeiss), (4) base holder, (5) glass container, (6) line lasers, (7) metal block, (8) laser power supply. Figure S2. Perfusion-based CUBIC cleared rat organs stained with propidium iodide. Bright field ...
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Myostatin genetic inactivation inhibits myogenesis by muscle-derived stem cells in vitro but not when implanted in the mdx mouse muscle
... Conclusions Our results show that MDSCs obtained from wild-type and Mst KO mice lacking myostatin express Oct 4 and other embryonic-like stem cell genes and appear similar in most features, except for the null or poor ...
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A reporter mouse for optical imaging of inflammation in mdx muscles
... other mdx models are available for non-invasive optical ...into mdx mice ...porter’ mouse accumulate luciferase signal over the life of the SJL mouse (dysferlin-deficient) by perpetually add- ...
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Preventing phosphorylation of dystroglycan ameliorates the dystrophic phenotype in mdx mouse
... Discussion 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 ...
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Preventing phosphorylation of dystroglycan ameliorates the dystrophic phenotype in mdx mouse
... Discussion 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 ...
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A comparison of the bone and growth phenotype of mdx, mdx:Cmah−/− and mdx:Utrn+/− murine models with the C57BL/10 wild-type mouse
... the mdx mouse resulted in reduced body fat (confirmed by DEXA body composition studies) and a consistently lower core body temperature, despite no overall body weight difference between the mdx and ...
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