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Skeletal muscle metabolism during prolonged exercise in Pompe disease

OBJECTIVE: Pompe disease (glycogenosis type II) is caused by lysosomal alpha-glucosidase deficiency, which leads to a block in intra-lysosomal glycogen breakdown. In spite of enzyme replacement therapy, Pompe disease continues to be a progressive metabolic myopathy. Considering the health benefits o...

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Detalles Bibliográficos
Autores principales: Preisler, Nicolai, Laforêt, Pascal, Madsen, Karen Lindhardt, Husu, Edith, Vissing, Christoffer Rasmus, Hedermann, Gitte, Galbo, Henrik, Lindberg, Christopher, Vissing, John
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Bioscientifica Ltd 2017
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8450668/
https://www.ncbi.nlm.nih.gov/pubmed/28490439
http://dx.doi.org/10.1530/EC-17-0042