Effect of deficient muscular glycogenolysis on extramuscular fuel production in exercise
Abstract
Hormonal, metabolic, and cardiovascular responses to 21 min of cycling in three saline- or glucose-infused men with McArdle's disease were compared with those of matched controls to elucidate whether mobilization of extramuscular fuel is enhanced to compensate for the lack of intramuscular glycogenolysis in patients with McArdle's disease. During exercise, all saline-infused patients compared with controls working at both the same absolute and at similar relative work rates had higher glucose production (31 +/- 7 vs. 19 +/- 5 and 26 +/- 4 mumol.min-1.kg-1) and utilization (34 +/- 8 vs. 22 +/- 2 and 28 +/- 4 mumol.min-1.kg-1); higher plasma glycerol (155 +/- 19 vs. 75 +/- 20 and 90 +/- 22 mumol/l), free fatty acids (487 +/- 175 vs. 295 +/- 47 and 202 +/- 52 mumol/l), growth hormone (7.7 +/- 2.8 vs. 2.6 +/- 1.1 and 3.6 +/- 3.4 mU/l), and cortisol (530 +/- 168 vs. 268 +/- 8 and 367 +/- 80 nmol/l), greater decrease in insulin (delta 57 +/- 4 vs. delta 11 +/- 8 and delta 11 +/- 23 pmol/l), and similar glucose concentrations. Furthermore, norepinephrine, epinephrine, and adrenocorticotropic hormone levels were higher and heart rate and cardiac output were higher during exercise in all patients than in controls at the same absolute work rate. Glucose infusion induced hyperglycemia and hyperinsulinemia in patients and inhibited the exercise-induced increases in glucose production, glycerol, free fatty acids, catecholamines, growth hormone, cortisol, and heart rate. In conclusion, feedback from metabolism in contracting muscle enhances hormonal responses and extramuscular substrate mobilization during exercise in McArdle's disease.
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Patients with McArdle's disease demonstrate a more pronounced mobilisation of extra-muscular carbohydrate sources during exercise compared with healthy controls, to compensate for the lack of intramuscular glycogenolysis.
“Moreover, it was observed that patients with McArdle’s disease demonstrate a more pronounced mobilisation of extra-muscular carbohydrate sources during exercise when compared with healthy controls, to compensate for the lack of intramuscular glycogenolysis [118].”
From Regulation of Energy Substrate Metabolism in Endurance Exercise (2021) · cites this paper as [118]
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- Authors
- Vissing, J., Lewis, S. F., Galbo, H., & Haller, R. G.
- Affiliations
- University of Copenhagen (DK)
- Dallas VA Medical Center (US)
- Venue
- Journal of Applied Physiology
- Publisher
- American Physiological Society
- Published
- 1992-05-01
- Volume
- 72
- Issue
- 5
- Pages
- 1773-1779
- ISSN
- 0161-7567 (ISSN-L)
- 8750-7587
- 1522-1601
- OpenAlex
- W85411597
- Cited by
- 67
- References
- 0
- Citations by year
- 2025: 2 · 2024: 2 · 2023: 2 · 2022: 1 · 2021: 1 · 2020: 1 · 2018: 1 · 2017: 3 · 2016: 3 · 2014: 3 · 2013: 2 · 2012: 2
- FWCI
- 1.38
- Type
- journal article
- Language
- en
- Open access
- closed
- Locations
- Journal of Applied Physiology (publishedVersion)
- PubMed (publishedVersion)
- Research at the University of Copenhagen (University of Copenhagen) (submittedVersion)
- Funders
- National Institutes of Health (5k04hl001581-02)
- National Heart, Lung, and Blood Institute (HL06296, HL 01581)
- Topics
- Health Sciences › Medicine › Rheumatology › Glycogen Storage Diseases and Myoclonus
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Molecular Biology › Metabolism, Diabetes, and Cancer
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Cell Biology › Muscle metabolism and nutrition
- Keywords
- McArdle disease, glycogen storage disease type V, glycogenolysis, glucose production, glucose utilization, plasma glycerol, free fatty acids, growth hormone, cortisol, insulin, catecholamines, cardiac output, exercise metabolism, glucose infusion
- MeSH terms
- Adult, Energy Metabolism, Glycogen, Glycogen Storage Disease Type V, Hemodynamics, Hormones, Humans, Lactates, Male, Muscles, Oxygen Consumption, Exercise, Lactic Acid
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