Results 231 to 240 of about 56,165 (274)

Canine X‐linked muscular dystrophy as an animal model of Duchenne muscular dystrophy: A review

American Journal of Medical Genetics, 1992
AbstractCanine 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, N J, Winand, D, Pradhan, N S, Moise, A, de Lahunta, J N, Kornegay, B J, Cooper   +6 more
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Erythrocytes from Animals with Genetic Muscular Dystrophy

Nature, 1973
EFFECTS 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, 1977
Two 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, 1989
Two 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, 2014
Congenital 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, Aryadnne L, Schactae, Jaime A, Soares, Letícia, Ventura, Viviane, Freiberger, Francielle, Mina, Diogo, Dominguini, Mariz, Vainzof, João, Quevedo   +8 more
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Animal models in therapeutic drug discovery for oculopharyngeal muscular dystrophy

Drug Discovery Today: Technologies, 2013
Oculopharyngeal 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, 2006
Oculopharyngeal 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, H, Hino, K, Araki, M, Takeya, M, Uchino, K, Yamamura   +5 more
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Altered tissue carnitine levels in animals with hereditary muscular dystrophy

Journal of the Neurological Sciences, 1978
Low levels of muscle carnitine have been found in patients with Duchenne dystrophy, a case possibly of Becker dystrophy, and limb-girdle syndrome as well as in patients with the recently described muscle carnitine deficiency syndrome. Tissues of the mouse, hamster, and chicken were analyzed to determine whether tissue carnitine levels were altered in ...
Borum, P R, Park, J H, Law, P K, Roelofs, R I   +3 more
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Animal models of muscular dystrophies.

Laboratory animal science, 1998
Recent advances in molecular biology have indicated that many mutant animal models of muscular dystrophy share common genetic and protein abnormalities similar to those of the human disease. The best example is a model of Duchenne muscular dystrophy (DMD), the mdx mouse.
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Muscular Dystrophy Gene Therapy in Small Animal Models

2009
Muscular dystrophies are inherited neuromuscular disorders characterized by progressive muscle loss and weakness. The morbidity and fatality associated with the diseases and a lack of effective treatment have prompted urgent search for novel therapeutics. Gene therapy is one of the frontiers. Currently, adeno-associated viral (AAV) vector-mediated gene
Chunping Qiao, Xiao Xiao
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