Skeletal muscle metabolism during prolonged exercise in Pompe disease
| Autor: | Pascal Laforêt, Christopher Lindberg, Henrik Galbo, Christoffer Rasmus Vissing, Edith Husu, John Vissing, Nicolai Preisler, Gitte Hedermann, Karen Lindhardt Madsen |
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| Přispěvatelé: | Rigshospitalet [Copenhagen], Copenhagen University Hospital, CHU Pitié-Salpêtrière [AP-HP], Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Institut de Myologie, Université Pierre et Marie Curie - Paris 6 (UPMC)-Commissariat à l'énergie atomique et aux énergies alternatives (CEA)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Association française contre les myopathies (AFM-Téléthon)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS), University of Gothenburg (GU), Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU), HAL UPMC, Gestionnaire |
| Jazyk: | angličtina |
| Rok vydání: | 2017 |
| Předmět: |
0301 basic medicine
medicine.medical_specialty Glycogenolysis skeletal muscle metabolism Endocrinology Diabetes and Metabolism Metabolic myopathy lcsh:Diseases of the endocrine glands. Clinical endocrinology 03 medical and health sciences chemistry.chemical_compound 0302 clinical medicine Endocrinology glycogenosis Internal medicine Internal Medicine medicine Respiratory exchange ratio Wasting [SDV.MHEP.EM] Life Sciences [q-bio]/Human health and pathology/Endocrinology and metabolism lcsh:RC648-665 Glycogen exercise business.industry Research Skeletal muscle Muscle weakness Pompe disease Enzyme replacement therapy [SDV.MHEP.EM]Life Sciences [q-bio]/Human health and pathology/Endocrinology and metabolism medicine.disease 3. Good health 030104 developmental biology medicine.anatomical_structure chemistry glycogen medicine.symptom business 030217 neurology & neurosurgery |
| Zdroj: | Endocrine Connections Endocrine Connections, BioScientifica Ltd., 2017, 6 (6), pp.384-394. ⟨10.1530/EC-17-0042⟩ Endocrine Connections, 2017, 6 (6), pp.384-394. ⟨10.1530/EC-17-0042⟩ Endocrine Connections, Vol 6, Iss 6, Pp 384-394 (2017) Preisler, N, Laforêt, P, Madsen, K L, Husu, E, Vissing, C R, Hedermann, G, Galbo, H, Lindberg, C & Vissing, J 2017, ' Skeletal muscle metabolism during prolonged exercise in Pompe disease ', Endocrine Connections, vol. 6, no. 6, pp. 384-394 . https://doi.org/10.1530/EC-17-0042 |
| ISSN: | 2049-3614 |
| DOI: | 10.1530/EC-17-0042⟩ |
| Popis: | 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 (VO2peak) 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, VO2peak was less than half of average control values; mean difference −1659 mL/min (CI: −2450 to −867, P = 0.001). However, the respiratory exchange ratio increased to >1.0 and lactate levels rose 5-fold in the patients, indicating significant glycolytic flux. In line with this, during submaximal exercise, the rates of oxidation (ROX) of carbohydrates and palmitate were similar between patients and controls (mean difference 0.226 g/min (CI: 0.611 to −0.078, P = 0.318) and mean difference 0.016 µmol/kg/min (CI: 1.287 to −1.255, P = 0.710), respectively). Conclusion Reflecting muscle weakness and wasting, Pompe disease is associated with markedly reduced maximal exercise capacity. However, glycogenolysis is not impaired in exercise. Unlike in other metabolic myopathies, skeletal muscle substrate use during exercise is normal in Pompe disease rendering exercise less complicated for e.g. medical or recreational purposes. |
| Databáze: | OpenAIRE |
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