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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Bibliographic Details
Main Authors: Nicolai Preisler, Pascal Laforêt, Karen Lindhardt Madsen, Edith Husu, Christoffer Rasmus Vissing, Gitte Hedermann, Henrik Galbo, Christopher Lindberg, John Vissing
Format: Article
Language:English
Published: Bioscientifica 2017-07-01
Series:Endocrine Connections
Subjects:
Online Access:http://www.endocrineconnections.com/content/6/6/384.full