title: ADAM12 alleviates the skeletal muscle pathology in mdx dystrophic mice.
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.
authors.affiliation: Institute of Molecular Pathology, University of Copenhagen, Denmark.
authors.forename: Pauliina
authors.initials: P
authors.lastname: Kronqvist
authors.forename: Nobuko
authors.initials: N
authors.lastname: Kawaguchi
authors.forename: Reidar
authors.initials: R
authors.lastname: Albrechtsen
authors.forename: Xiufeng
authors.initials: X
authors.lastname: Xu
authors.forename: Henrik Daa
authors.initials: HD
authors.lastname: Schrøder
authors.forename: Behzad
authors.initials: B
authors.lastname: Moghadaszadeh
authors.forename: Finn Cilius
authors.initials: FC
authors.lastname: Nielsen
authors.forename: Camilla
authors.initials: C
authors.lastname: Fröhlich
authors.forename: Eva
authors.initials: E
authors.lastname: Engvall
authors.forename: Ulla M
authors.initials: UM
authors.lastname: Wewer
mesh_terms: D051722:ADAM Proteins; D000072199:ADAM12 Protein; D000818:Animals; D006801:Humans; D007249:Inflammation / Q000473:pathology; D008565:Membrane Proteins / Q000235:genetics / Q000502:physiology*; D008666:Metalloendopeptidases / Q000235:genetics / Q000502:physiology*; D051379:Mice; D018101:Mice, Inbred mdx; D008822:Mice, Transgenic; D018482:Muscle, Skeletal / Q000473:pathology*; D009136:Muscular Dystrophies / Q000473:pathology*; D009336:Necrosis
chemical_list: D008565:Membrane Proteins; D051722:ADAM Proteins; D000072199:ADAM12 Protein; C000606480:ADAM12 protein, human; D008666:Metalloendopeptidases