A 3 months mild functional test regime does not affect disease parameters in young mdx mice

Neuromuscul Disord. 2010 Apr;20(4):273-80. doi: 10.1016/j.nmd.2010.02.004. Epub 2010 Mar 21.

Abstract

To assess the effect of potential therapeutic agents in dystrophic mice it is useful to have a functional test regime that does not affect the natural disease progression of mdx mice with dystrophinopathy. We determined the effect of a 12 week test regime consisting of fore limb grip strength, rotarod analysis and two and four limb hanging wire tests on the disease progression of 4-week-old mdx mice. Mice performed the different functional tests on consecutive days on a weekly basis. No difference was found in serum creatine kinase levels between functionally active and sedentary mice. The percentage of fibrotic/necrotic areas assessed in a semi-automated way with colour deconvolution of skeletal muscles, heart and diaphragm did not vary within muscles or between groups, nor did the gene expression levels of disease-related genes. We conclude that this test regime may be suitable for short-term functional evaluation of therapeutic approaches in the mdx mouse.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Age Factors
  • Animals
  • Creatine Kinase / analysis
  • Creatine Kinase / blood
  • Disability Evaluation*
  • Disease Models, Animal
  • Fibrosis / drug therapy
  • Fibrosis / metabolism
  • Fibrosis / physiopathology
  • Gene Expression Regulation / genetics
  • Heart / drug effects
  • Heart / physiopathology
  • Image Processing, Computer-Assisted
  • Male
  • Mice
  • Mice, Inbred mdx
  • Motor Activity / drug effects
  • Motor Activity / genetics
  • Muscle Fibers, Skeletal / metabolism
  • Muscle Fibers, Skeletal / pathology
  • Muscle Proteins / genetics
  • Muscle Strength / drug effects
  • Muscle Strength / genetics
  • Muscle, Skeletal / drug effects
  • Muscle, Skeletal / metabolism
  • Muscle, Skeletal / physiopathology
  • Muscular Dystrophy, Duchenne / diagnosis*
  • Muscular Dystrophy, Duchenne / drug therapy
  • Muscular Dystrophy, Duchenne / physiopathology*
  • Myocardium / metabolism
  • Neurologic Examination / methods
  • Outcome Assessment, Health Care / methods*
  • Predictive Value of Tests
  • Recovery of Function / drug effects
  • Recovery of Function / genetics
  • Severity of Illness Index*
  • Time Factors

Substances

  • Muscle Proteins
  • Creatine Kinase