Impaired performance of skeletal muscle in alpha-glucosidase knockout mice.

  title={Impaired performance of skeletal muscle in alpha-glucosidase knockout mice.},
  author={Reinout P. Hesselink and Marchel Gorselink and Gert Schaart and Anton J M Wagenmakers and Joep H. J. Kamphoven and Arnold J. J. Reuser and Ger J. van der Vusse and Maarten Robert Drost},
  journal={Muscle & nerve},
  volume={25 6},
Glycogen 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… CONTINUE READING


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