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Science 11 June 1971:
Vol. 172. no. 3988, pp. 1143 - 1145
DOI: 10.1126/science.172.3988.1143
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Articles

Duchenne Muscular Dystrophy: Functional Ischemia Reproduces Its Characteristic Lesions

J. R. Mendell 1, W. King Engel 1, and E. C. Derrer 1

1 National Institute of Neurological Diseases and Stroke, Bethesda, Maryland 20014

The highly characteristic early and midstage histological lesions of Duchenne dystrophy were reproduced experimentally in the rat by the combination of a vascular abnormality, aortic ligation, which does not affect the structure of the intramuscular blood vessels, and the humoral vasoactive substance 5-hydroxytryptamine. Neither ligation nor injection of 5-hydroxytryptamine alone causes changes in the muscle fibers. This result establishes the possibility of a similar combined mechanism for a nonstructural ischemia pathogenesis in Duchenne dystrophy. The proposed pathogenesis is contrary to the generally held idea that the cause is an intrinsic abnormality of muscle fiber metabolism.


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