title: Bone marrow transplantation improves outcome in a mouse model of congenital muscular dystrophy.
abstract: We examined whether pathogenesis in dystrophin-deficient (mdx) mice and laminin-alpha2-deficient (dy) mice is ameliorated by bone marrow transplantation (BMT). Green fluorescent protein (GFP) mice were used as donors. In mdx mice, BMT failed to produce any significant differences in muscle pathology, although some GFP-positive fibers with restored dystrophin expression were observed. In contrast, in the dy mice, BMT led to a significant increase in lifespan and an increase in growth rate, muscle strength, and respiratory function. We conclude that BMT improved outcome in dy mice but not mdx mice.
authors.affiliation: Division of Neurology, Department of Internal Medicine, Kawasaki Medical School, 577 Matsushima, Kurashiki-City, Okayama 701-0192, Japan.
authors.forename: Hiroki
authors.initials: H
authors.lastname: Hagiwara
authors.forename: Yutaka
authors.initials: Y
authors.lastname: Ohsawa
authors.forename: Shoji
authors.initials: S
authors.lastname: Asakura
authors.forename: Tatsufumi
authors.initials: T
authors.lastname: Murakami
authors.forename: Takanori
authors.initials: T
authors.lastname: Teshima
authors.forename: Yoshihide
authors.initials: Y
authors.lastname: Sunada
mesh_terms: D000818:Animals; D001835:Body Weight; D016026:Bone Marrow Transplantation; D004195:Disease Models, Animal; D007797:Laminin / Q000235:genetics; D051379:Mice; D008810:Mice, Inbred C57BL; D018101:Mice, Inbred mdx; D018345:Mice, Knockout; D018482:Muscle, Skeletal / Q000473:pathology / Q000503:physiopathology; D009136:Muscular Dystrophies / Q000151:congenital / Q000473:pathology / Q000601:surgery*; D012119:Respiration; D015996:Survival Rate; D016896:Treatment Outcome
chemical_list: D007797:Laminin; C102645:laminin alpha 2