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An Autoradiographic Study of Muscular Dystrophy, Motor Neuron Disease and Charcot-Marie-Tooth Disease

Published online by Cambridge University Press:  03 July 2018

George Monckton  and
Halyna Marusyk
George Monckton*
Affiliation:
University of Alberta, Edmonton, Alberta, Canada
Halyna Marusyk
Affiliation:
University of Alberta, Edmonton, Alberta, Canada
*
9101 Clinical Sciences Building, University of Alberta, Edmonton, Alberta, Canada T6G 1S2.
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Summary

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The autoradiographic findings using tritiated leucine are described in muscle biopsy material from five patients with progressive muscular dystrophy (P.M.D.), three with motor neuron disease (M.N.D.) and four with Charcot-Marie-Tooth disease (C.M.T.). In progressive muscular dystrophy there is a marked increase in uptake of leucine into cytoplasmic proteins and precursors, and reduced incorporation into structural protein. In Charcot-Marie Tooth disease muscle there is a significantly increased uptake into cytoplasmic elements and a normal uptake into structural protein. In motor neuron disease the uptake into cytoplasmic elements appears normal but is reduced into structural proteins.

The abnormal uptake in C.M.T. could be explained as a product of regenerative efforts associated with reinnervation. However, the abnormal uptake may represent the primary effects of gene action in the muscle, as seems probable in progressive muscular dystrophy.

Résumé

Résumé

Nous décrivons les observations autoradiographiques avec leucine tritiée de biopsies musculaires provenant de cinq patients atteints de dystrophie musculaire progressive (P.M.D.), de trois patients atteints de maladies du neurone moteur (M.N.D.), et de quatre patients atteints de Charcot-Marie-Tooth (C.M.T ). Les résultats montrent que dans la dystrophie musculaire progressive il y a une augmentation marquée de la captation de la leucine dans les protéines cytoplasmiques et leurs précurseurs, réduisant d’autant l’incorporation à même la protéine structurale. Dans le muscle de patients atteints de la maladie de Charcot-Marie-Tooth, il y a une augmentation significative de la captation par les éléments cytoplasmiques et une captation normale au niveau de la protéine structurale. Pour ce qui est des maladies du neurone moteur, la captation dans les éléments cytoplasmiques semble normale, mais elle est diminuée dans les protéines structurales.

La captation anormale dans le C.M.T. pourrait s’expliquer comme le résultat d’une effort régénération associé à une réinnervation. Toutefois, la captation anormale pourrait aussi représenter les premiers effets de l’action génétique au niveau du muscle, tout comme probablement dans la dystrophie musculaire progressive.

Type
Research Article
Information
Canadian Journal of Neurological Sciences , Volume 4 , Issue 1 , February 1977 , pp. 25 - 29
Copyright
Copyright © Canadian Neurological Sciences Federation 1977

References

Brimijoin, S., Capek, P. and Dyck, P. J. (1973). Axonal transport of Dopamine hydroxylase by human sural nerves in vitro. Science 180: 1295–1297.CrossRefGoogle ScholarPubMed
Coers, C. (1976). Changes in Motor Unit pattern in the Charcot-Marie peroneal muscular atrophy as compared to spinal muscular atrophy In press IV. Journees Internationales De Pathologie Neuro-Musculaire.Google Scholar
Engel, W. K. (1967b). Muscle biopsies in neuromuscular diseases. Pediatric Clinics of America. 24: 963–995.CrossRefGoogle Scholar
Haase, G. R. and Shy, G. M. (1960). Pathological changes in muscle biopsies from patients with perineal muscular atrophy. Brain 83: 631–637.CrossRefGoogle Scholar
Kitchin, S. E. and Watts, D. C. (1973). Comparison of the turnover patterns of total and individual proteins in normal mice and those with hereditary muscular dystrophy. Biochem. J. 136: 1017–1028.CrossRefGoogle ScholarPubMed
McComas, A. J., Sica, R. E. P. and Currie, S. (1970). Muscular dystrophy: evidence for a neural factor. Nature, 226: 1263–1264.CrossRefGoogle Scholar
McComas, A. J., Sica, R. E. P. and Campbell, M. J. (1971a). “Sick” motoneurons. A unifying concept of muscle disease. Lancet, 1: 321–325.CrossRefGoogle ScholarPubMed
McComas, A. J., Sica, R. E. P. and Currie, S. (1971d). An electrophysiological study of Duchenne dystrophy. J. Neurol. Neurosurg. Psychiat. 34: 461–468.CrossRefGoogle ScholarPubMed
McComas, A. J., Sica, R. E. P. and Upton, A. R. M. (1974b). Multiple muscle analysis of motor units in muscular dystrophy. Archives of Neurology, 30: 249–251.CrossRefGoogle ScholarPubMed
Monckton, G. and Marusyk, H. (1975). 3H leucine incorporation in myofibrils of normal and dystrophic mouse skeletal muscle. Canad. J. of Neurol. Sci. 2: 1–4.CrossRefGoogle ScholarPubMed
Monckton, G. and Marusyk, H. (1976). Myofibrillar incorporation of 3H (G) L-leucine in progressive muscular dystrophy and motor neuron disease. Neurol. 26: 234–237 CrossRefGoogle ScholarPubMed
Monckton, G. and Nihei, T. (1969). A correlation of histology and amino acid incorporation studies in Duchenne muscular dystrophy. Neurol. 19: 415–418.CrossRefGoogle ScholarPubMed
Nihei, T. Tataryn, D. and Filipenko, C. (1971). The reduction of myosin synthesis in muscle of dystrophic mice. In: Basic Research in Myology p. 204, Exerpta Medica. Amsterdam.Google Scholar
Pleasure, D. E., Mishler, K. C. and Engel, W. K. (1969). Axonal transport of proteins in experimental neuropathies. Science. 166: 524–525.CrossRefGoogle ScholarPubMed
Rogers, A. W. (1969). Techniques of autoradiography New York, Elsevier Publishing Co. Google Scholar
Szent-Gyorgyi, A. (1947). Chemistry of muscle contraction. New York, Academic Press.Google Scholar
Thomson, W. H. S. and Sweetin, J. C. (1974). The neurogenic and myogenic hypothesis in human (Duchenne) muscular dystrophy Nature 249: 151–152.CrossRefGoogle Scholar
Tomé, F. M. S. and Fardeau, M. (1976). Muscle and nerve biopsies studies in Charcot-Marie-Tooth disease. In press, IV Journees Internationales De Pathologie Neuro-Musculaire.Google Scholar