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Metabolism of selenium in skeletal muscle and liver of mice with genetic muscular dystrophy

Journal Article · · Proc. Soc. Exp. Biol. Med.; (United States)
Genetic muscular dystrophy is characterized by progressive muscular weakness and atrophy and necrosis of the skeletal muscle. In several aspects, the disease in genetically dystrophic mice is thought to resemble that in human beings. For a variety of species, dietary selenium deficiency can result in a state of nutritional muscular dystrophy which resembles the symptoms of inherited muscular dystrophy. However, nutritional muscular dystrophy has not been observed in animals given adequate vitamin E regardless of selenium status. Furthermore, it has been shown that genetic muscular dystrophy cannot be reversed by the addition of selenium to the diet. Several investigators have shown that glutathione peroxidase (glutathione:hydrogenperoxidase oxidoreductase, EC 1.11.1.9) activity is increased in mice and chickens with inherited muscular dystrophy. It is well documented that this enzyme requires selenium for activity and that its activity decreases markedly in response to dietary selenium deficiency . Thus, because the glutathione peroxidase activity is increased in dystrophic muscle, the metabolic basis for muscular dystrophy does not appear to be related to selenium deficiency. This increased activity of glutathione peroxidase in dystrophic muscle suggests that both selenium and enzyme protein concentrations are increased. The elevated enzyme level in the dystrophic muscle may be explained by an alteration in the rate of selenium retention and absorption which permits selenium accumulation to abnormally high concentrations. The present studies were performed to determine if such an alteration in selenium metabolism occurs in muscle from genetically dystrophic mice.
Research Organization:
Univ. of Tennessee, Oak Ridge
DOE Contract Number:
W-7405-ENG-26
OSTI ID:
5260461
Journal Information:
Proc. Soc. Exp. Biol. Med.; (United States), Journal Name: Proc. Soc. Exp. Biol. Med.; (United States) Vol. 160; ISSN PSEBA
Country of Publication:
United States
Language:
English