Muscle glycogen unavailability and fat oxidation rate during exercise: Insights from McArdle disease

Carbohydrate availability affects fat metabolism during exercise; however, the effects of complete muscle glycogen unavailability on maximal fat oxidation (MFO) rate remain unknown. Our purpose was to examine the MFO rate in patients with McArdle disease, comprising an inherited condition caused by...

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Bibliographic Details
Published in:The Journal of physiology 2023-02, Vol.601 (3), p.551-566
Main Authors: Rodriguez‐Lopez, Carlos, Santalla, Alfredo, Valenzuela, Pedro. L, Real‐Martínez, Alberto, Villarreal‐Salazar, Mónica, Rodriguez‐Gomez, Irene, Pinós, Tomàs, Ara, Ignacio, Lucia, Alejandro
Format: Article
Language:English
Subjects:
Adipose tissue
Adipose Tissue - metabolism
anaplerotic
Animal models
Animals
Calorimetry
Carbohydrate metabolism
Exercise
Exercise Test
Fat metabolism
fatty acids
Female
Glycogen
Glycogen - metabolism
glycogen depletion
Glycogen Storage Disease Type V - metabolism
glycogen store disease
lactate
Lipid metabolism
Lipids
Male
Metabolism
Mice
muscle fatigue
Muscle, Skeletal - physiology
Oxidation
Oxidation-Reduction
Oxygen Consumption - physiology
Skeletal muscle
substrate oxidation
tricarboxylic acid cycle
Workloads
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Summary:Carbohydrate availability affects fat metabolism during exercise; however, the effects of complete muscle glycogen unavailability on maximal fat oxidation (MFO) rate remain unknown. Our purpose was to examine the MFO rate in patients with McArdle disease, comprising an inherited condition caused by complete blockade of muscle glycogen metabolism, compared to healthy controls. Nine patients (three women, aged 36 ± 12 years) and 12 healthy controls (four women, aged 40 ± 13 years) were studied. Several molecular markers of lipid transport/metabolism were also determined in skeletal muscle (gastrocnemius) and white adipose tissue of McArdle (Pygm p.50R*/p.50R*) and wild‐type male mice. Peak oxygen uptake (V̇O2peak${\dot V_{{{\rm{O}}_{\rm{2}}}{\rm{peak}}}$), MFO rate, the exercise intensity eliciting MFO rate (FATmax) and the MFO rate‐associated workload were determined by indirect calorimetry during an incremental cycle‐ergometer test. Despite having a much lower V̇O2peak${\dot V_{{{\rm{O}}_{\rm{2}}}{\rm{peak}}}$ (24.7 ± 4 vs. 42.5 ± 11.4 mL kg−1 min−1, respectively; P 
ISSN:0022-3751
1469-7793
DOI:10.1113/JP283743