| Effect of changes in fat availability on exercise capacity in McArdle disease. | |
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MedLine Citation:
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PMID: 19506137 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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BACKGROUND: The major fuel for exercising muscle at low exercise intensities is fat. OBJECTIVE: To investigate the role of fat metabolism in McArdle disease (also known as glycogen storage disease type V), an inborn error of muscle glycogenolysis, by manipulating free fatty acid availability for oxidation during exercise. DESIGN: Randomized, placebo-controlled, crossover trial. SETTING: Hospitalized care. PATIENTs: Ten patients (8 men and 2 women) with McArdle disease. INTERVENTIONS: Patients cycled at a constant workload corresponding to 70% of their maximum oxygen consumption. In random order and on separate days, patients received nicotinic acid (a known blocker of lipolysis) to decrease the availability of free fatty acids or 20% Intralipid infusion to increase free fatty acid availability during exercise. Results were compared with placebo (isotonic sodium chloride solution infusion) and glucose infusion trials. MAIN OUTCOME MEASURES: Exercise tolerance was assessed by heart rate response to exercise during different infusions. RESULTS: Free fatty acid levels more than tripled by Intralipid infusion and were halved by nicotinic acid administration. Heart rate was significantly higher during exercise in the Intralipid infusion and nicotinic acid trials compared with the placebo and glucose infusion trials, an effect that was observed before and after the patients had experienced the second wind phenomenon. CONCLUSIONS: Lipids are an important source of fuel for exercising muscle in McArdle disease, but maximal rates of fat oxidation seem limited and cannot be increased above physiologically normal rates during exercise. This limitation is probably caused by a metabolic bottleneck in the tricarboxylic acid cycle due to impaired glycolytic flux in McArdle disease. Therapies aimed at enhancing fat use in McArdle disease should be combined with interventions targeting expansion of the tricarboxylic acid cycle. |
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Authors:
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Susanne T Andersen; Tina D Jeppesen; Tanja Taivassalo; Marie-Louise Sveen; Katja Heinicke; Ronald G Haller; John Vissing |
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Publication Detail:
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Type: Journal Article; Randomized Controlled Trial; Research Support, Non-U.S. Gov't |
Journal Detail:
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Title: Archives of neurology Volume: 66 ISSN: 1538-3687 ISO Abbreviation: Arch. Neurol. Publication Date: 2009 Jun |
Date Detail:
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Created Date: 2009-06-09 Completed Date: 2009-08-03 Revised Date: - |
Medline Journal Info:
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Nlm Unique ID: 0372436 Medline TA: Arch Neurol Country: United States |
Other Details:
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Languages: eng Pagination: 762-6 Citation Subset: AIM; IM |
Affiliation:
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Neuromuscular Research Unit, Department of Neurology, and Copenhagen Muscle Research Center, University of Copenhagen, Rigshospitalet, Copenhagen, Denmark. |
Export Citation:
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| MeSH Terms | |
Descriptor/Qualifier:
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Adipose Tissue
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drug effects*,
metabolism Adult Antilipemic Agents / administration & dosage Citric Acid Cycle / drug effects, physiology Cross-Over Studies Energy Metabolism / drug effects, physiology Exercise Tolerance / drug effects*, physiology Fatty Acids, Nonesterified / blood Female Glycogen Storage Disease Type V / drug therapy*, metabolism, physiopathology Glycolysis / drug effects, physiology Humans Lipid Mobilization / drug effects*, physiology Male Muscle Strength / drug effects, physiology Muscle, Skeletal / drug effects*, metabolism Niacin / administration & dosage* Oxygen Consumption / drug effects, physiology Physical Fitness / physiology Placebos Treatment Outcome Young Adult |
| Chemical | |
Reg. No./Substance:
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0/Antilipemic Agents; 0/Fatty Acids, Nonesterified; 0/Placebos; 59-67-6/Niacin |
From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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