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Pathogenesis of progressive muscular dystrophy: studies on free radical metabolism in an animal model
Author(s) -
Ohta K.,
Muzuno Y.
Publication year - 1988
Publication title -
acta neurologica scandinavica
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.967
H-Index - 95
eISSN - 1600-0404
pISSN - 0001-6314
DOI - 10.1111/j.1600-0404.1988.tb05880.x
Subject(s) - muscular dystrophy , glutathione peroxidase , superoxide dismutase , catalase , pathogenesis , glutathione reductase , medicine , phosphofructokinase , endocrinology , lactate dehydrogenase , metabolism , biochemistry , glycolysis , biology , enzyme , chemistry
— Evidence to suggest the presence of abnormal metabolism of oxygen free radicals in progressive muscular dystrophy is presented using an animal model. In the superficial pectoral muscles of dystrophic chickens, enzyme activities regulating the metabolism of oxygen free radicals, i.e., catalase, superoxide dismutases and glutathione peroxidase, were significantly elevated within 1 week of hatching. Activities of related enzymes, i.e., glutathione reductase, glucose‐6‐phosphate dehydrogenase, 6‐phosphogluconate dehydrogenase were also elevated. In contrast, the specific activity of phosphofructokinase, the rate‐limiting enzyme of the glycolytic pathway, was normal during the first 4‐week period. These results suggest that there is an increased turnover of oxygen free radicals in the dystrophic muscle. This concept appears important in a further investigation of the pathogenesis and treatment of progressive muscular dystrophies.