Exercise testing and prescription in patients with inborn errors of muscle energy metabolism
Skeletal muscle is a dynamic organ requiring tight regulation of energy metabolism in order
to provide bursts of energy for effective function. Several inborn errors of muscle energy …
to provide bursts of energy for effective function. Several inborn errors of muscle energy …
Muscle glycogen unavailability and fat oxidation rate during exercise: Insights from McArdle disease
C Rodriguez‐Lopez, A Santalla… - The Journal of …, 2023 - Wiley Online Library
Carbohydrate availability affects fat metabolism during exercise; however, the effects of
complete muscle glycogen unavailability on maximal fat oxidation (MFO) rate remain …
complete muscle glycogen unavailability on maximal fat oxidation (MFO) rate remain …
[HTML][HTML] Mitochondrial Dysfunction in Glycogen Storage Disorders (GSDs)
K Mishra, O Kakhlon - Biomolecules, 2024 - mdpi.com
Glycogen storage disorders (GSDs) are a group of inherited metabolic disorders
characterized by defects in enzymes involved in glycogen metabolism. Deficiencies in …
characterized by defects in enzymes involved in glycogen metabolism. Deficiencies in …
Acute ketone supplementation in the absence of muscle glycogen utilization: Insights from McArdle disease
Background & aims Ketone supplementation is gaining popularity. Yet, its effects on
exercise performance when muscle glycogen cannot be used remain to be determined …
exercise performance when muscle glycogen cannot be used remain to be determined …
Aerobic capacity and muscle proteome: Insights from a mouse model
We explored the association between aerobic capacity (AC) and the skeletal muscle
proteome of McArdle (n= 10) and wild‐type (n= 8) mice, as models of intrinsically 'low'and …
proteome of McArdle (n= 10) and wild‐type (n= 8) mice, as models of intrinsically 'low'and …
[HTML][HTML] Development of Continuum of Care for McArdle disease: A practical tool for clinicians and patients
McArdle disease (glycogen storage disease type V; GSDV) is a rare genetic disease caused
by the inability to break down glycogen in skeletal muscle due to a deficiency in …
by the inability to break down glycogen in skeletal muscle due to a deficiency in …
[HTML][HTML] Breaking Barriers: Strength Training Feasibility in McArdle Syndrome-A systematic review
The practice of physical fitness training plays a crucial role in the management of McArdle
disease (MD), a metabolic myopathy characterized by myophosphorylase deficiency, which …
disease (MD), a metabolic myopathy characterized by myophosphorylase deficiency, which …
Late Presentation of McArdle's Disease Mimicking Polymyalgia Rheumatica: A Case Report and Review of the Literature
M Elghobashy, U Pohl… - Case Reports in …, 2025 - Wiley Online Library
McArdle disease or glycogen storage disease Type V is a genetic condition caused by
PYGM gene mutations leading to exercise intolerance and fatigability. The condition most …
PYGM gene mutations leading to exercise intolerance and fatigability. The condition most …
[HTML][HTML] Low aerobic capacity in McArdle disease: A role for mitochondrial network impairment?
M Villarreal-Salazar, A Santalla, A Real-Martínez… - Molecular …, 2022 - Elsevier
Background McArdle disease is caused by myophosphorylase deficiency and results in
complete inability for muscle glycogen breakdown. A hallmark of this condition is muscle …
complete inability for muscle glycogen breakdown. A hallmark of this condition is muscle …
An experiment of nature links muscle glycogen unavailability with very high fat oxidation rates despite low aerobic fitness.
J Nielsen - Journal of Physiology, 2023 - search.ebscohost.com
Muscle glycogen unavailability and fat oxidation rate during exercise: Insights from McArdle
disease. Keywords: exercise; fat metabolism; glycogen; McArdle disease EN exercise fat …
disease. Keywords: exercise; fat metabolism; glycogen; McArdle disease EN exercise fat …