Abstract

Enzyme activity patterns of selected key enzymes in energy-supplying metabolism were determined in muscle biopsy specimens of patients withmyotonia congenita (autosomal recessive inherited) and in skeletal muscle of rats with 20,25-diazacholesterol-induced myotonia. A close correlation exists in muscles of normal humans between the activity levels of hexokinase and citrate synthase. This correlation is disturbed both in human and experimental myotonia and consists in an increase of the relative hexokinase activity in the human and a decrease in the rat.

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