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Myofibrillar incorporation of ³H (G) L-leucine in progressive muscular dystrophy and motor neuron disease

From the Department of Medicine, University of Alberta, Edmonton, Alberta, Canada.

A study of the incorporation of hydrogen 3 (³H) leucine into skeletal muscle in patients with human muscular dystrophy and motor neuron disease showed uptake in cytoplasm and myofibrils. The techniques used were light and electron microscopy and autoradiography before and after glycerination (Szent-Gyorgyi). The results indicated an increase of ³H leucine uptake into cytoplasm of the dystrophic muscle and a marked drop in incorporation into structural protein. In the patients with motor neuron disease, the normal incorporation into cytoplasm was seen, with reduced uptake into structural protein. These results showed a different protein synthesis pattern in dystrophic and denervated muscle.

Dr. Monckton's address is Neurology Room 9-103B, Clinical Sciences Bldg., University of Alberta, 11282 - 84th Ave., Edmonton, Alberta, Canada T6G 2G3.

This work was supported by a grant from the Muscular Dystrophy Association of Canada.

Received for publication July 11, 1975.