Fat and Sugar Metabolism During Exercise in Patients With Metabolic Myopathy

Inclusion Criteria: * Men and women with genetically and/or biochemically verified LCHAD Deficiency * Healthy controls needs to be healthy to be included, evaluated by the investigator. Exclusion Criteria: * Competing conditions at risk of compromising the results of the study. * Pregnancy or breastfeeding * Cardiac or pulmonary disease contraindicating peak exercise testing or strenuous exercise. * Inability to understand the purpose of the trial or corporate for the conduction of the experiments. * Veins that are too difficult to puncture for blood sampling or insertion of intravenous catheters, evaluated by the investigator * Moderate to severe muscle weakness, that prevents the subject completing 10 minutes of cycle-ergometry exercise at 60-70 % of VO2peak, evaluated by the investigator. * Participation in other trials that may interfere with the results. * Intake of medications that may interfere with the results or may compromise exercise performance, as evaluated by the investigators. * Donation of blood within 30 days prior to conduction of the tests on test day 1.