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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

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