ADAM12 alleviates the skeletal muscle pathology in mdx dystrophic mice

Pauliina Kronqvist, Nobuko Kawaguchi, Reidar Albrechtsen, Xiufeng Xu, Henrik Daa Schrøder, Behzad Moghadaszadeh, Finn Cilius Nielsen, Camilla Frohlich, Eva Engvall, Ulla M Wewer

54 Citations (Scopus)

Abstract

Muscular dystrophy is characterized by muscle degeneration and insufficient regeneration and replacement of muscle fibers by connective tissue. New therapeutic strategies directed toward various forms of muscular dystrophy are needed to preserve muscle mass and promote regeneration. In this study we examined the role of the transmembrane ADAM12, a disintegrin and metalloprotease, which is normally associated with development and regeneration of skeletal muscle. We demonstrate that ADAM12 overexpression in the dystrophin-deficient mdx mice alleviated the muscle pathology in these animals, as evidenced by less muscle cell necrosis and inflammation, lower levels of serum creatine kinase, and less uptake of Evans Blue dye into muscle fibers. These studies demonstrate that ADAM12 directly or indirectly contributes to muscle cell regeneration, stability, and survival.
Original languageEnglish
JournalAmerican Journal of Pathology
Volume161
Issue number5
Pages (from-to)1535-40
Number of pages6
ISSN0002-9440
DOIs
Publication statusPublished - 1 Nov 2002

Keywords

  • ADAM Proteins
  • Animals
  • Humans
  • Inflammation
  • Membrane Proteins
  • Metalloendopeptidases
  • Mice
  • Mice, Inbred mdx
  • Mice, Transgenic
  • Muscle, Skeletal
  • Muscular Dystrophies
  • Necrosis

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