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Endpoint measures in the mdx mouse relevant for muscular dystrophy pre-clinical studies.

Abstract
Loss of mobility influences the quality of life for patients with neuromuscular diseases. Common measures of mobility and chronic muscle damage are the six-minute walk test and serum creatine kinase. Despite extensive pre-clinical studies of therapeutic approaches, characterization of these measures is incomplete. To address this, a six-minute ambulation assay, serum creatine kinase, and myoglobinuria were investigated for the mdx mouse, a dystrophinopathy mouse model commonly used in pre-clinical studies. mdx mice ambulated shorter distances than normal controls, a disparity accentuated after mild exercise. An asymmetric pathophysiology in mdx mice was unmasked with exercise, and peak measurements of serum creatine kinase and myoglobinuria were identified. Our data highlights the necessity to consider asymmetric pathology and timing of biomarkers when testing potential therapies for muscular dystrophy.
AuthorsYvonne M Kobayashi, Erik P Rader, Robert W Crawford, Kevin P Campbell
JournalNeuromuscular disorders : NMD (Neuromuscul Disord) Vol. 22 Issue 1 Pg. 34-42 (Jan 2012) ISSN: 1873-2364 [Electronic] England
PMID22154712 (Publication Type: Journal Article, Research Support, American Recovery and Reinvestment Act, Research Support, N.I.H., Extramural, Research Support, Non-U.S. Gov't)
CopyrightCopyright © 2011 Elsevier B.V. All rights reserved.
Chemical References
  • Creatine Kinase
Topics
  • Animals
  • Creatine Kinase (blood)
  • Disease Models, Animal
  • Male
  • Mice
  • Mice, Inbred C57BL
  • Mice, Inbred mdx
  • Muscle, Skeletal (pathology, physiopathology)
  • Muscular Dystrophy, Duchenne (diagnosis, pathology, physiopathology)
  • Myoglobinuria (urine)
  • Physical Conditioning, Animal (physiology)

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