Patients with McArdle disease have blocked muscle glycogen breakdown due to an inherited defect myophosphorylase. This causes exercise intolerance with muscle pain, contractures and rhabdomyolysis. McArdle patients cannot increase fat oxidation to fully compensate for the energy deficiency due to a slow turnover in the tricarboxylic acid cycle (TCA). Metabolism of the 7-carbon fatty acid, triheptanoin, generates acetyl-CoA and propionyl-CoA, which enter the TCA and can therefore potentially boost fat oxidation in McArdle patients.

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