Fat and carbohydrate metabolism during exercise in late-onset Pompe disease

Article ID	Journal	Published Year	Pages	File Type
10833503	Molecular Genetics and Metabolism	2012	7 Pages	PDF

Abstract

âº Metabolism was examined with stable isotopes and indirect calorimetry. âº Peak oxidative capacity was severely reduced to less than half normal in patients. âº Fat and carbohydrate oxidation during sub-maximal exercise was normal in patients. âº Glucose supplementation did not improve exercise capacity in patients. âº This indicates that exercise intolerance in Pompe disease is structurally related.

Keywords

RER FFA GSD ROX RPE GAA VO2peak Free fatty acids Stable isotopes Glycogen storage disease Ratings of perceived exertion Wpeak body mass index BMI Skeletal muscle metabolism Metabolic myopathy rate of appearance rate of disappearance Respiratory exchange ratio Glycogen storage disease type II exercise Glycogen

Related Topics

Life Sciences Biochemistry, Genetics and Molecular Biology Biochemistry

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Fat and carbohydrate metabolism during exercise in late-onset Pompe disease

Authors

Nicolai Preisler, Pascal Laforet, Karen Lindhardt Madsen, Regitze SÃ¸lling Hansen, Zoltan Lukacs, Mette Cathrine Ãrngreen, Arnaud Lacour, John Vissing,