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FEBS Lett. 2006 Aug 7;580(18):4463-8. Epub 2006 Jul 14.

Bone marrow transplantation improves outcome in a mouse model of congenital muscular dystrophy.

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1
Division of Neurology, Department of Internal Medicine, Kawasaki Medical School, 577 Matsushima, Kurashiki-City, Okayama 701-0192, Japan.

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.

PMID:
16859688
DOI:
10.1016/j.febslet.2006.07.015
[Indexed for MEDLINE]
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