Bone marrow transplantation improves outcome in a mouse model of congenital muscular dystrophy.
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.
Hagiwara
Hiroki
H
Division of Neurology, Department of Internal Medicine, Kawasaki Medical School, 577 Matsushima, Kurashiki-City, Okayama 701-0192, Japan.
Ohsawa
Yutaka
Y
Asakura
Shoji
S
Murakami
Tatsufumi
T
Teshima
Takanori
T
Sunada
Yoshihide
Y
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
D007797:Laminin; C102645:laminin alpha 2