Impaired muscle spindle function in murine models of muscular dystrophy
نویسندگان
چکیده
منابع مشابه
Lung and respiratory muscle function in limb girdle muscular dystrophy.
BACKGROUND Pulmonary involvement is frequently observed in patients with limb girdle muscular dystrophy and occurs early in the disease. The aim of this study was to establish the prevalence of pulmonary dysfunction; the type of dysfunction; and any correlation between patient age, disease duration, or limb weakness and lung or respiratory muscle dysfunction. METHODS Twenty patients with stri...
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BACKGROUND The muscular dystrophies target muscle groups differentially. In mouse models of muscular dystrophy, notably the mdx model of Duchenne Muscular Dystrophy, the diaphragm muscle shows marked fibrosis and at an earlier age than other muscle groups, more reflective of the histopathology seen in human muscular dystrophy. METHODS Using a mouse model of limb girdle muscular dystrophy, the...
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Duchenne muscular dystrophy (DMD) is an X-linked genetic disorder characterized by progressive muscle degeneration. Mutations in the DMD gene result in the absence of dystrophin, a protein required for muscle strength and stability. Currently, there is no cure for DMD. Since murine models are relatively easy to genetically manipulate, cost effective, and easily reproducible due to their short g...
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A trial of sodium aurothiomalate as an antiproteinase drug in the treatment of murine muscular dystrophy is reported. A blind controlled comparison of high (25 microgram/10 g body weight) and low dose gold (5 microgram/10 g body weight) with saline-injected control animals was made, all injections being given three times weekly. The body weights and functional ability of the mice were assessed ...
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Disruption of dystrophin or the sarcoglycans within the dystrophin protein complex leads to muscular dystro-phy. The dystrophin complex is enriched in myofibers and concentrated into costameres, rib-like structures in the plasma membrane. In the absence of dystrophin, the plasma membrane becomes fragile with reduced stiffness and increased leakiness, and exposing dystro-phin-deficient muscle to...
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ژورنال
عنوان ژورنال: The Journal of Physiology
سال: 2020
ISSN: 0022-3751,1469-7793
DOI: 10.1113/jp278563