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- W2023089966 abstract "To determine whether impaired exercise capacity in neutral lipid storage disease with myopathy is solely caused by muscle weakness or whether a defect in energy metabolism (blocked fat oxidation) may also play a role.We studied a 37-year-old woman with neutral lipid storage disease with myopathy, who cycled while lipid oxidation was assessed using U-(13)C palmitate tracer dilution technique. The effect of a glucose infusion during exercise was also studied.Neuromuscular research unit.The exercise-induced increase in fat oxidation was virtually abolished in the patient. Treatment with intravenous glucose infusion improved maximal oxygen uptake from 23 to 27 mL × kg(-1) × min(-1), and maximal workload from 75 to 100 W.These results demonstrate that in addition to fixed weakness, neutral lipid storage disease with myopathy is also characterized by a profound block in fat oxidation, which limits exercise tolerance." @default.
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- W2023089966 date "2012-04-01" @default.
- W2023089966 modified "2023-10-13" @default.
- W2023089966 title "Blocked Muscle Fat Oxidation During Exercise in Neutral Lipid Storage Disease" @default.
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- W2023089966 doi "https://doi.org/10.1001/archneurol.2011.631" @default.
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