Fat metabolism during exercise in patients with McArdle disease

M. C. Ørngreen, T. D. Jeppesen, S. Tvede Andersen, T. Taivassalo, S. Hauerslev, N. Preisler, R. G. Haller, G. Van Hall, J. Vissing

Research output: Contribution to journalArticle

33 Citations (Scopus)

Abstract

OBJECTIVE:: It is known that muscle phosphorylase deficiency restricts carbohydrate utilization, but the implications for muscle fat metabolism have not been studied. We questioned whether patients with McArdle disease can compensate for the blocked muscle glycogen breakdown by enhancing fat oxidation during exercise. METHODS:: We studied total fat oxidation by indirect calorimetry and palmitate turnover by stable isotope methodology in 11 patients with McArdle disease and 11 healthy controls. Cycle exercise at a constant workload of 50% to 60% of maximal oxygen uptake capacity was used to evaluate fatty acid oxidation (FAO) in the patients. Healthy controls were exercised at the same absolute workload. RESULTS:: We found that palmitate oxidation and disposal, total fat oxidation, and plasma levels of palmitate and total free fatty acids (FFAs) were significantly higher, whereas total carbohydrate oxidation was lower, during exercise in patients with McArdle disease vs healthy controls. We found augmented fat oxidation with the onset of a second wind, but further increases in FFA availability, as exercise continued, did not result in further increases in FAO. CONCLUSION:: These results indicate that patients with McArdle disease have exaggerated fat oxidation during prolonged, low-intensity exercise and that increased fat oxidation may be an important mechanism of the spontaneous second wind. The fact that increasing availability of free fatty acids with more prolonged exercise did not increase fatty acid oxidation suggests that blocked glycogenolysis may limit the capacity of fat oxidation to compensate for the energy deficit in McArdle disease.

Original languageEnglish (US)
Pages (from-to)718-724
Number of pages7
JournalNeurology
Volume72
Issue number8
DOIs
StatePublished - Jul 24 2009

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Glycogen Storage Disease Type V
Fats
Exercise
Palmitates
Nonesterified Fatty Acids
Fatty Acids
Workload
Carbohydrates
Glycogenolysis
Muscles
Indirect Calorimetry
Glycogen
Isotopes
Oxygen

ASJC Scopus subject areas

  • Clinical Neurology

Cite this

Ørngreen, M. C., Jeppesen, T. D., Andersen, S. T., Taivassalo, T., Hauerslev, S., Preisler, N., ... Vissing, J. (2009). Fat metabolism during exercise in patients with McArdle disease. Neurology, 72(8), 718-724. https://doi.org/10.1212/01.wnl.0000343002.74480.e4

Fat metabolism during exercise in patients with McArdle disease. / Ørngreen, M. C.; Jeppesen, T. D.; Andersen, S. Tvede; Taivassalo, T.; Hauerslev, S.; Preisler, N.; Haller, R. G.; Van Hall, G.; Vissing, J.

In: Neurology, Vol. 72, No. 8, 24.07.2009, p. 718-724.

Research output: Contribution to journalArticle

Ørngreen, MC, Jeppesen, TD, Andersen, ST, Taivassalo, T, Hauerslev, S, Preisler, N, Haller, RG, Van Hall, G & Vissing, J 2009, 'Fat metabolism during exercise in patients with McArdle disease', Neurology, vol. 72, no. 8, pp. 718-724. https://doi.org/10.1212/01.wnl.0000343002.74480.e4
Ørngreen MC, Jeppesen TD, Andersen ST, Taivassalo T, Hauerslev S, Preisler N et al. Fat metabolism during exercise in patients with McArdle disease. Neurology. 2009 Jul 24;72(8):718-724. https://doi.org/10.1212/01.wnl.0000343002.74480.e4
Ørngreen, M. C. ; Jeppesen, T. D. ; Andersen, S. Tvede ; Taivassalo, T. ; Hauerslev, S. ; Preisler, N. ; Haller, R. G. ; Van Hall, G. ; Vissing, J. / Fat metabolism during exercise in patients with McArdle disease. In: Neurology. 2009 ; Vol. 72, No. 8. pp. 718-724.
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