Interleukin-15 administration improves diaphragm muscle pathology and function in dystrophic mdx mice

Am J Pathol. 2005 Apr;166(4):1131-41. doi: 10.1016/S0002-9440(10)62333-4.

Abstract

Interleukin (IL)-15, a cytokine expressed in skeletal muscle, has been shown to have muscle anabolic effects in vitro and to slow muscle wasting in rats with cancer cachexia. Whether IL-15 has therapeutic potential for diseases such as Duchenne muscular dystrophy (DMD) is unknown. We examined whether IL-15 administration could ameliorate the dystrophic pathology in the diaphragm muscle of the mdx mouse, an animal model for DMD. Four weeks of IL-15 treatment improved diaphragm strength, a highly significant finding because respiratory function is a mortality predictor in DMD. Enhanced diaphragm function was associated with increased muscle fiber cross-sectional area and decreased collagen infiltration. IL-15 administration was not associated with changes in T-cell populations or alterations in specific components of the ubiquitin proteasome pathway. To determine the effects of IL-15 on myofiber regeneration, muscles of IL-15-treated and untreated wild-type mice were injured myotoxically, and their functional recovery was assessed. IL-15 had a mild anabolic effect, increasing fiber cross-sectional area after 2 and 6 days but not after 10 days. Our findings demonstrate that IL-15 administration improves the pathophysiology of dystrophic muscle and highlight a possible therapeutic role for IL-15 in the treatment of neuromuscular disorders especially in which muscle wasting is indicated.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Animals
  • Diaphragm / drug effects*
  • Diaphragm / pathology
  • Gene Expression
  • Humans
  • Immunohistochemistry
  • Interleukin-15 / therapeutic use*
  • Macrophages / drug effects
  • Male
  • Mice
  • Mice, Inbred mdx
  • Muscle Fibers, Skeletal / drug effects*
  • Muscle Fibers, Skeletal / pathology
  • Muscular Dystrophy, Animal / drug therapy*
  • Regeneration / drug effects
  • Reverse Transcriptase Polymerase Chain Reaction
  • T-Lymphocytes / drug effects
  • Ubiquitin / metabolism
  • Ubiquitin-Conjugating Enzymes / metabolism

Substances

  • Interleukin-15
  • Ubiquitin
  • Ubiquitin-Conjugating Enzymes