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Utrophin-Dystrophin-Deficient Mice as a Model for Duchenne Muscular Dystrophy
The absence of dystrophin at the muscle membrane leads to Duchenne muscular dystrophy (DMD), a severe muscle-wasting disease that is inevitably fatal in early adulthood. In contrast,Expand
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Animal Models for Muscular Dystrophy Show Different Patterns of Sarcolemmal Disruption
Genetic defects in a number of components of the dystrophin–glycoprotein complex (DGC) lead to distinct forms of muscular dystrophy. However, little is known about how alterations in the DGC areExpand
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Cardiomyopathic features associated with muscular dystrophy are independent of dystrophin absence in cardiovasculature
The loss of dystrophin results in skeletal muscle degeneration and cardiomyopathy in patients with Duchenne muscular dystrophy. In skeletal muscle, dystrophin strengthens the myofiber membrane byExpand
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Contraction-induced injury to single permeabilized muscle fibers from mdx, transgenic mdx, and control mice.
Muscle fibers of mdx mice that lack dystrophin are more susceptible to contraction-induced injury, particularly when stretched. In contrast, transgenic mdx (tg-mdx) mice, which overexpressExpand
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Selective loss of sarcolemmal nitric oxide synthase in Becker muscular dystrophy
Becker muscular dystrophy is an X-linked disease due to mutations of the dystrophin gene. We now show that neuronal-type nitric oxide synthase (nNOS), an identified enzyme in the dystrophin complex,Expand
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Skeletal muscle-specific expression of a utrophin transgene rescues utrophin-dystrophin deficient mice
Duchenne muscular dystrophy (DMD) is a progressive muscle wasting disease usually resulting in death of patients by their early twenties1. In contrast, mice lacking dystrophin (Dmdmdx), appearExpand
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Forced expression of dystrophin deletion constructs reveals structure- function correlations
Dystrophin plays an important role in skeletal muscle by linking the cytoskeleton and the extracellular matrix. The amino terminus of dystrophin binds to actin and possibly other components of theExpand
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Consequences of the combined deficiency in dystrophin and utrophin on the mechanical properties and myosin composition of some limb and respiratory muscles of the mouse
The mechanical properties and the myosin isoform composition were studied in three isolated muscles (EDL, soleus, diaphragm) of mutant mice lacking both dystrophin and utrophin (dko). They wereExpand
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A quantitative study of bioenergetics in skeletal muscle lacking utrophin and dystrophin
Muscle energetics and function were investigated in the hindlimb of mice lacking dystrophin (mdx), utrophin and dystrophin (utr-dys) and controls (C57Bl/10) using 31P and 1H magnetic resonanceExpand
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Expression of full-length and truncated dystrophin mini-genes in transgenic mdx mice.
Duchenne and Becker muscular dystrophy are caused by defects in the dystrophin gene, and are candidates for treatment by gene therapy. We have shown previously that overexpression of a full-lengthExpand
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