Acute ketone supplementation in the absence of muscle glycogen utilization: Insights from McArdle disease.

Autor: Valenzuela PL; Physical Activity and Health Research Group ('PaHerg'), Research Institute of Hospital '12 de Octubre' ('imas12'), Madrid, Spain; Department of Systems Biology, University of Alcalá, Madrid, Spain. Electronic address: pedrol.valenzuela@uah.es., Santalla A; Department of Sport and Computer Science, Section of Physical Education and Sports, Faculty of Sport, Universidad Pablo de Olavide, Sevilla, Spain; EVOPRED Research Group, Universidad Europea de Canarias, Tenerife, Spain., Alejo LB; Physical Activity and Health Research Group ('PaHerg'), Research Institute of Hospital '12 de Octubre' ('imas12'), Madrid, Spain; Faculty of Sport Sciences, Universidad Europea de Madrid, Madrid, Spain., Bustos A; Faculty of Sport Sciences, Universidad Europea de Madrid, Madrid, Spain., Ozcoidi LM; Hospital Reina Sofía de Tudela, Servicio Navarro de Salud, Navarra, Spain., Castellote-Bellés L; Department of Clinical Biochemistry, Laboratoris Clínics, Hospital Universitari Vall d'Hebron, Barcelona, Spain., Ferrer-Costa R; Department of Clinical Biochemistry, Laboratoris Clínics, Hospital Universitari Vall d'Hebron, Barcelona, Spain., Villarreal-Salazar M; Mitochondrial and Neuromuscular Disorders Unit, Vall d'Hebron Institut de Recerca, Universitat Autònoma de Barcelona, Barcelona, Spain; Spanish Network for Biomedical Research in Rare Diseases (CIBERER), U723, Madrid, Spain., Morán M; Spanish Network for Biomedical Research in Rare Diseases (CIBERER), U723, Madrid, Spain; Mitochondrial and Neuromuscular Diseases Laboratory, Research Institute of Hospital 12 de Octubre (imas12), Madrid, Spain., Barranco-Gil D; Faculty of Sport Sciences, Universidad Europea de Madrid, Madrid, Spain., Pinós T; Mitochondrial and Neuromuscular Disorders Unit, Vall d'Hebron Institut de Recerca, Universitat Autònoma de Barcelona, Barcelona, Spain; Spanish Network for Biomedical Research in Rare Diseases (CIBERER), U723, Madrid, Spain. Electronic address: tomas.pinos@vhir.org., Lucia A; Faculty of Sport Sciences, Universidad Europea de Madrid, Madrid, Spain.
Jazyk: angličtina
Zdroj: Clinical nutrition (Edinburgh, Scotland) [Clin Nutr] 2024 Mar; Vol. 43 (3), pp. 692-700. Date of Electronic Publication: 2024 Jan 26.
DOI: 10.1016/j.clnu.2024.01.026
Abstrakt: Background & Aims: Ketone supplementation is gaining popularity. Yet, its effects on exercise performance when muscle glycogen cannot be used remain to be determined. McArdle disease can provide insight into this question, as these patients are unable to obtain energy from muscle glycogen, presenting a severely impaired physical capacity. We therefore aimed to assess the effects of acute ketone supplementation in the absence of muscle glycogen utilization (McArdle disease).
Methods: In a randomized cross-over design, patients with an inherited block in muscle glycogen breakdown (i.e., McArdle disease, n = 8) and healthy controls (n = 7) underwent a submaximal (constant-load) test that was followed by a maximal ramp test, after the ingestion of a placebo or an exogenous ketone ester supplement (30 g of D-beta hydroxybutyrate/D 1,3 butanediol monoester). Patients were also assessed after carbohydrate (75 g) ingestion, which is currently considered best clinical practice in McArdle disease.
Results: Ketone supplementation induced ketosis in all participants (blood [ketones] = 3.7 ± 0.9 mM) and modified some gas-exchange responses (notably increasing respiratory exchange ratio, especially in patients). Patients showed an impaired exercise capacity (-65 % peak power output (PPO) compared to controls, p < 0.001) and ketone supplementation resulted in a further impairment (-11.6 % vs. placebo, p = 0.001), with no effects in controls (p = 0.268). In patients, carbohydrate supplementation resulted in a higher PPO compared to ketones (+21.5 %, p = 0.001) and a similar response was observed vs. placebo (+12.6 %, p = 0.057).
Conclusions: In individuals who cannot utilize muscle glycogen but have a preserved ability to oxidize blood-borne glucose and fat (McArdle disease), acute ketone supplementation impairs exercise capacity, whereas carbohydrate ingestion exerts the opposite, beneficial effect.
(Copyright © 2024 Elsevier Ltd and European Society for Clinical Nutrition and Metabolism. All rights reserved.)
Databáze: MEDLINE
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