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ADAM12 alleviates the skeletal muscle pathology in mdx dystrophic mice.
Tekijät: Kronqvist, Kawaguchi, Albrechtsen, Xu, Schrøder, Moghadaszadeh, Nielsen, Fröhlich, Engvall, Wewer
Julkaisuvuosi: 2002
Journal: American Journal of Pathology
Tietokannassa oleva lehden nimi: The American journal of pathology
Lehden akronyymi: Am J Pathol
Vuosikerta: 161
Numero: 5
Aloitussivu: 1535
Lopetussivu: 40
Sivujen määrä: 6
ISSN: 0002-9440
DOI: https://doi.org/10.1016/S0002-9440(10)64431-8
Tiivistelmä
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.
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.