Impaired performance of skeletal muscle in alpha-glucosidase knockout mice
| Autor: | Ger J. van der Vusse, Arnold J. J. Reuser, Gert Schaart, Anton J. M. Wagenmakers, Reinout P. Hesselink, Marchel Gorselink, Maarten R. Drost, Joep H. J. Kamphoven |
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| Přispěvatelé: | Bewegingswetenschappen, Fysiologie, Humane Biologie, RS: CARIM School for Cardiovascular Diseases, RS: NUTRIM School of Nutrition and Translational Research in Metabolism, Pediatrics, Clinical Genetics, Soft Tissue Biomech. & Tissue Eng. |
| Rok vydání: | 2002 |
| Předmět: |
medicine.medical_specialty
Phosphocreatine Physiology Muscle Fibers Skeletal Actinin Isometric exercise Biology Desmin Cellular and Molecular Neuroscience Mice Adenosine Triphosphate Inosine Monophosphate Physiology (medical) Internal medicine medicine Myocyte Animals Muscle Skeletal Mice Knockout Muscle Weakness Glycogen Storage Disease Type II Body Weight Skeletal muscle Muscle weakness alpha-Glucosidases Adenosine Monophosphate Adenosine Diphosphate medicine.anatomical_structure Endocrinology Tetanic contraction Neurology (clinical) Stress Mechanical medicine.symptom Glucan 1 4-alpha-Glucosidase Glycogen Muscle contraction Muscle Contraction |
| Zdroj: | Muscle & Nerve, 25(6), 873-883. Wiley Muscle & Nerve, 25, 873-883. John Wiley & Sons Inc. Muscle and nerve, 25(6), 873-883. Wiley |
| ISSN: | 0148-639X |
| Popis: | Department of Movement Sciences, Cardiovascular Research Institute, Maastricht University, P.O. Box 616, 6200 MD Maastricht, The Netherlands. Reinout.Hesselink@BW.Unimaas.nlGlycogen storage disease type II (GSD II) is an inherited progressive muscle disease in which lack of functional acid alpha-glucosidase (AGLU) results in lysosomal accumulation of glycogen. We report on the impact of a null mutation of the acid alpha-glucosidase gene (AGLU(-/-)) in mice on the force production capabilities, contractile mass, oxidative capacity, energy status, morphology, and desmin content of skeletal muscle. Muscle function was assessed in halothane-anesthetized animals, using a recently designed murine isometric dynamometer. Maximal torque production during single tetanic contraction was 50% lower in the knockout mice than in wild type. Loss of developed torque was found to be disproportionate to the 20% loss in muscle mass. During a series of supramaximal contraction, fatigue, expressed as percentile decline of developed torque, did not differ between AGLU(-/-) mice and age-matched controls. Muscle oxidative capacity, energy status, and protein content (normalized to either dry or wet weight) were not changed in knockout mice compared to control. Alterations in muscle cell morphology were clearly visible. Desmin content was increased, whereas alpha-actinin was not. As the decline in muscle mass is insufficient to explain the degree in decline of mechanical performance, we hypothesize that the large clusters of noncontractile material present in the cytoplasm hamper longitudinal force transmission, and hence muscle contractile function. The increase in muscular desmin content is most likely reflecting adaptations to altered intracellular force transmission. |
| Databáze: | OpenAIRE |
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