Bone marrow transplantation improves outcome in a mouse model of congenital muscular dystrophy
Bone marrow transplantation improves outcome in a mouse model of congenital muscular dystrophy
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DOI：
10.1016/j.febslet.2006.07.015
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发表时间：
2006-08-07
期刊：
影响因子：
3.5
通讯作者：
Sunada, Yoshihide
中科院分区：
文献类型：
--
作者：
Hagiwara, Hiroki;Ohsawa, Yutaka;Sunada, Yoshihide
我们研究了骨髓移植（BMT）是否能改善肌营养不良蛋白缺陷（mdx）小鼠和层粘连蛋白α 2缺陷（dy）小鼠的发病机制。使用绿色荧光蛋白（GFP）小鼠作为供体。在mdx小鼠中，BMT未能产生肌肉病理学的任何显着差异，尽管观察到一些具有恢复的肌营养不良蛋白表达的GFP阳性纤维。相比之下，在dy小鼠中，BMT导致寿命显着增加，生长速度，肌肉力量和呼吸功能增加。我们得出结论，骨髓移植改善了dy小鼠的预后，但对mdx小鼠无影响。(c)2006年欧洲生物化学学会联合会。Elsevier B.V.出版，保留所有权利。
We examined whether pathogenesis in dystrophin-deficient (mdx) mice and laminin-alpha 2-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. (c) 2006 Federation of European Biochemical Societies. Published by Elsevier B.V. All rights reserved.