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Canine X‐linked muscular dystrophy as an animal model of Duchenne muscular dystrophy: A review
American Journal of Medical Genetics, 1992AbstractCanine X‐linked muscular dystrophy is a spontaneously occurring, progressive, degenerative myopathy of dogs that is clinically and pathologically similar to Duchenne muscular dystrophy in man. The molecular basis for the disease has been shown to be a lack of dystrophin, the protein product of the Duchenne muscular dystrophy gene.
B A, Valentine +6 more
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Cardiomyopathy in animal models of muscular dystrophy
Current Opinion in Cardiology, 2001Arrhythmia and cardiomyopathy frequently accompany muscular dystrophy. In the last year, the cardiovascular consequences of muscular dystrophy gene mutations have been established through studies of murine models. These models have highlighted the potential role of primary defects in cardiac muscle as well as those secondary cardiovascular outcomes ...
A, Heydemann, M T, Wheeler, E M, McNally
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Animal models of muscular dystrophy
2001Abstract Scientists have employed animal models for research into muscular dystrophy over the last two decades. These models have been used for pathophysiological analysis as well as for biochemical studies. The clinical symptoms and pathological features observed in animal models are somewhat different from those in human patients ...
Satoru Noguchi, Yukiko K Hayashi
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Muscular Dystrophy in Man and Animals.
Archives of Neurology, 1963The emergent interest in diseases of muscle has been accompanied by a remarkable profusion of books. Within the past four years, the following volumes have been published: the Association for Research in Nervous and Mental DiseaseSymposium on Neuromuscular Diseases, the Proceedings of the Second International Myasthenia Gravis Conference, the second ...
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Erythrocytes from Animals with Genetic Muscular Dystrophy
Nature, 1973EFFECTS of congenital muscular dystrophy include changes in processes associated with membrane systems of muscle1,2. Moreover, there is evidence that genetic dystrophy in mice, which appears to be a suitable model for the human disease3, is associated with alteration of membrane events occurring in other tissue as well4–8. For example, we have reported
Morse, P, Howland, J
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[Human myopathy and animal muscular dystrophy].
Acta zoologica et pathologica Antverpiensia, 1977Two hereditary muscular dystrophies similar to human progressive muscular dystrophy (P.M.D. Duchenne type) have been isolated in animals, one in mouse, the other in chicken. The decrease in the activity of glycogenolytic enzymes is similar to that observed in denervated muscle.
G, Schapira, J C, Dreyfus, F, Schapira
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Animal modes of Duchenne and Becker muscular dystrophy
British Medical Bulletin, 1989Two animal models have been shown to be related to Duchenne and Becker muscular dystrophy at the molecular level. The mdx mouse is characterized by early onset of muscle degeneration and very mild clinical disease. The disease is minimally progressive and fibrosis of muscle is absent.
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Behavioral Responses in Animal Model of Congenital Muscular Dystrophy 1D
Molecular Neurobiology, 2014Congenital muscular dystrophies 1D (CMD1D) present a mutation on the LARGE gene and are characterized by an abnormal glycosylation of α-dystroglycan (α-DG), strongly implicated as having a causative role in the development of central nervous system abnormalities such as cognitive impairment seen in patients.
Clarissa M, Comim +8 more
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Animal models in therapeutic drug discovery for oculopharyngeal muscular dystrophy
Drug Discovery Today: Technologies, 2013Oculopharyngeal muscular dystrophy (OPMD) is a late onset disease which affects specific muscles. No pharmacological treatments are currently available for OPMD. In recent years, genetically tractable models of OPMD – Drosophila and Caenorhabditis elegans – have been generated.
Chartier, A., Simonelig, M.
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Animal model of oculopharyngeal muscular dystrophy.
Acta myologica : myopathies and cardiomyopathies : official journal of the Mediterranean Society of Myology, 2006Oculopharyngeal muscular dystrophy (OPMD) is a late-onset polyalanine disorder characterized clinically by progressive ptosis, dysphagia, and limb weakness and pathological hallmarked by unique intranuclear inclusions in the muscles. It is caused by heterozygous expansion of a 10-alanine stretch to 12-17 alanine residues in the N-terminus of the poly(A)
E, Uyama +5 more
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