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Bone marrow transplantation improves outcome in a mouse model of congenital muscular dystrophy
Author(s) -
Hagiwara Hiroki,
Ohsawa Yutaka,
Asakura Shoji,
Murakami Tatsufumi,
Teshima Takanori,
Sunada Yoshihide
Publication year - 2006
Publication title -
febs letters
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.593
H-Index - 257
eISSN - 1873-3468
pISSN - 0014-5793
DOI - 10.1016/j.febslet.2006.07.015
Subject(s) - dystrophin , muscular dystrophy , duchenne muscular dystrophy , pathogenesis , mdx mouse , bone marrow , transplantation , green fluorescent protein , medicine , bone marrow transplantation , endocrinology , pathology , biology , gene , biochemistry
We examined whether pathogenesis in dystrophin‐deficient ( mdx ) mice and laminin‐α2‐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.