The DOI matches its Crossref record; 2 of the 2 references it cites were found in a registry or an index.
Increased PFK activity and GLUT4 protein content in McArdle’s disease
Abstract
Inborn errors of metabolism represent an opportunity to conduct studies in order to understand compensatory adaptations to a defective metabolic pathway. We evaluated the molecular and biochemical adaptations in substrate metabolism (glycolysis, electron transport chain, tricarboxylic acid cycle, β‐oxidation) in response to myophosphorylase deficiency in skeletal muscle from 13 patients with McArdle's disease (MD) and 13 age‐matched controls. MD muscle had higher phosphofructokinase protein content and activity as well as glucose transporter 4 (GLUT4) protein content and lower GLUT4 mRNA content than controls. At the protein level, skeletal muscle adaptations suggest an augmented glucose transport and glycolytic flux as a compensatory metabolic strategy to a chronic absence of muscle glycogen phosphorylase. These results support previous findings of increased glucose uptake during exercise and alleviation of symptoms with oral sucrose in patients with MD. Muscle Nerve, 2007
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“potentially due to the fact that the higher dose likely impaired their disease up-regulated phosphofructokinase (PFK) activity [218,219]”
From Creatine monohydrate supplementation for older adults and clinical populations (2025) · cites this paper as [218]
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Gibala, M. J., Little, J. P., Van Essen, M., Wilkin, G. P., Burgomaster, K. A., Safdar, A., Raha, S., & Tarnopolsky, M. A. (2006) · The Journal of Physiology · doi:10.1113/jphysiol.2006.112094 · Cited by 1,082
Self-citationEffect of deficient muscular glycogenolysis on extramuscular fuel production in exercise
Vissing, J., Lewis, S. F., Galbo, H., & Haller, R. G. (1992) · Journal of Applied Physiology · doi:10.1152/jappl.1992.72.5.1773 · Cited by 67
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van Hall, G. (2015) · Sports Medicine · doi:10.1007/s40279-015-0394-8 · Cited by 77
Creatine monohydrate supplementation for older adults and clinical populations
Candow, D. G., Ostojic, S. M., Chilibeck, P. D., Longobardi, I., Gualano, B., Tarnopolsky, M. A., Wallimann, T., Moriarty, T., Kreider, R. B., Forbes, S. C., et al. (2025) · Journal of the International Society of Sports Nutrition · doi:10.1080/15502783.2025.2534130 · Cited by 43
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Metadata
- Authors
- Robertshaw, H. A., Raha, S., Kaczor, J. J., & Tarnopolsky, M. A.
- Venue
- Muscle & Nerve
- Publisher
- Wiley
- Published
- 2008-03-19
- Volume
- 37
- Issue
- 4
- Pages
- 431-437
- ISSN
- 0148-639X
- 1097-4598
- Cited by
- 16
- References
- 2
- Language
- en
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