Myopathy in McArdle's Syndrome

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Abstract
IN normal muscle, the principal sources of energy during exercise are glucose and fatty acids. The proportion of energy derived from each of these substrates depends on the intensity and duration of exercise.1 Amino acids are known to serve two important functions of muscle metabolism: they furnish a substrate for muscle protein synthesis and provide amino groups for the production of the gluconeogenetic amino acids alanine and glutamine.2 Evidence is now available that certain amino acids, in particular the branched-chain amino acids, may also serve as a direct source of muscle energy.2 In McArdle's syndrome (myophosphorylase deficiency), muscle glycogenolysis is . . .

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