Skeletal muscle metabolism during prolonged exercise in Pompe disease.

ABSTRACT:

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 of exercise, it is important in Pompe disease to acquire more information about muscle substrate use during exercise.

Methods

Seven adults with Pompe disease were matched to a healthy control group (1:1). We determined (1) peak oxidative capacity (VO_2peak) and (2) carbohydrate and fatty acid metabolism during submaximal exercise (33 W) for 1 h, using cycle-ergometer exercise, indirect calorimetry and stable isotopes.

Results

In the patients, VO_2peak was

SUBMITTER: Preisler N 

PROVIDER: S-EPMC8450668 | biostudies-literature | 2017 Aug

REPOSITORIES: biostudies-literature