Mdx Mice
Mostrando 25-36 de 81 artigos, teses e dissertações.
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25. Distribuição dos receptores de acetilcolina e terminais nervosos na junção neuromuscular de fibras musculares regeneradas
Mdx mice have deficiency of dystrophin and cycles of muscle fiber regeneration. Changes in the distribution of acetylcholine receptors have been reported at the neuromuscular junction of mdx and may be a consequence of muscle fiber regeneration. At the present, we verified whether the distribution of receptors is still altered in long-term regenerated muscle
Publicado em: 2004
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26. Impaired regeneration of dystrophin-deficient muscle fibers is caused by exhaustion of myogenic cells
Duchenne muscular dystrophy is one of the most devastating myopathies. Muscle fibers undergo necrosis and lose their ability to regenerate, and this may be related to increased interstitial fibrosis or the exhaustion of satellite cells. In this study, we used mdx mice, an animal model of Duchenne muscular dystrophy, to assess whether muscle fibers lose their
Brazilian Journal of Medical and Biological Research. Publicado em: 2002-06
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27. Recovery of induced mutations for X chromosome-linked muscular dystrophy in mice.
We have used elevated levels of plasma creatine phosphokinase activity to identify muscular dystrophy phenotypes in mice and to screen the progeny of chemical mutagen-treated male mice for X chromosome-linked muscular dystrophy mutations. We were not successful in identifying heterozygous carriers of these induced muscular dystrophy mutations in greater than
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28. Severe cardiomyopathy in mice lacking dystrophin and MyoD
The mdx mouse, a mouse model of Duchenne muscular dystrophy, carries a loss-of-function mutation in dystrophin, a component of the membrane-associated dystrophin–glycoprotein complex. Unlike humans, mdx mice rarely display cardiac abnormalities and exhibit dystrophic changes only in a small number of heavily used skeletal muscle groups. By contrast, mdx:My
The National Academy of Sciences.
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29. Myotubes from transgenic mdx mice expressing full-length dystrophin show normal calcium regulation.
A lack of dystrophin results in muscle degeneration in Duchenne muscular dystrophy. Dystrophin-deficient human and mouse muscle cells have higher resting levels of intracellular free calcium ([Ca2+]i) and show a related increase in single-channel open probabilities of calcium leak channels. Elevated [Ca2+]i results in high levels of calcium-dependent proteol
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30. Matrix metalloproteinase-9 inhibition ameliorates pathogenesis and improves skeletal muscle regeneration in muscular dystrophy
Duchenne muscular dystrophy (DMD) is a fatal X-linked genetic disorder of skeletal muscle caused by mutation in dystrophin gene. Although the degradation of skeletal muscle extracellular matrix, inflammation and fibrosis are the common pathological features in DMD, the underlying mechanisms remain poorly understood. In this study, we have investigated the ro
Oxford University Press.
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31. Contractile properties of skinned muscle fibres from young and adult normal and dystrophic (mdx) mice.
1. Single muscle fibres were enzymatically isolated from the soleus and extensor digitorum longus (EDL) muscles of genetically dystrophic mdx and normal (C57BL/10) mice aged 3-6 or 17-23 weeks. 2. Fibres of both muscles were chemically skinned with the non-ionic detergent Triton X-100 (2% v/v). Ca(2+)- and Sr(2+)-activated contractile responses were recorded
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32. Overexpression of the cytotoxic T cell GalNAc transferase in skeletal muscle inhibits muscular dystrophy in mdx mice
Duchenne muscular dystrophy (DMD) is a congenital X-linked myopathy caused by lack of dystrophin protein expression. In DMD, the expression of many dystrophin-associated proteins (DAPs) is reduced along the sarcolemmal membrane, but the same proteins remain concentrated at the neuromuscular junction where utrophin, a dystrophin homologue, is expressed [Matsu
National Academy of Sciences.
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33. Malformed mdx myofibers have normal cytoskeletal architecture yet altered EC coupling and stress-induced Ca2+ signaling
Skeletal muscle function is dependent on its highly regular structure. In studies of dystrophic (dy/dy) mice, the proportion of malformed myofibers decreases after prolonged whole muscle stimulation, suggesting that the malformed myofibers are more prone to injury. The aim of this study was to assess morphology and to measure excitation-contraction (EC) coup
American Physiological Society.
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34. Similarity of ATP-dependent K+ channels in skeletal muscle fibres from normal and mutant mdx mice.
1. ATP-dependent K+ (KATP) channels were studied in fibres isolated from flexor digitorum brevis and interosseal skeletal muscles of normal and mutant mdx mice using the patch clamp technique in the presence of asymmetrical K+ concentrations (5 mM K+ in the pipette and in vivo intracellular [K+] or 145 mM K+ at the cytoplasmic face). 2. In cell-attached patc
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35. Myonuclear apoptosis in dystrophic mdx muscle occurs by perforin-mediated cytotoxicity.
Myonuclear apoptosis is an early event in the pathology of dystrophin-deficient muscular dystrophy in the mdx mouse. However, events that initiate apoptosis in muscular dystrophy are unknown, and whether elimination of apoptosis can ameliorate subsequent muscle wasting remains a major question. We have tested the hypothesis that cytotoxic T-lymphocytes initi
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36. Imatinib attenuates skeletal muscle dystrophy in mdx mice
Duchenne-Meryon muscular dystrophy (DMD) is the most common and lethal genetic muscle disease. Ameliorating muscle necrosis, inflammation, and fibrosis represents an important therapeutic approach for DMD. Imatinib, an antineoplastic agent, demonstrated antiinflammatory and antifibrotic effects in liver, kidney, lung, and skin of various animal models. This
The Federation of American Societies for Experimental Biology.