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ADAM12过表达并不能改善层粘连蛋白α2缺陷型肌营养不良小鼠的预后。

ADAM12 overexpression does not improve outcome in mice with laminin alpha2-deficient muscular dystrophy.

作者信息

Guo Ling T, Shelton G Diane, Wewer Ulla M, Engvall Eva

机构信息

The Burnham Institute, 10901 North Torrey Pines Road, La Jolla, CA 92037, USA.

出版信息

Neuromuscul Disord. 2005 Nov;15(11):786-9. doi: 10.1016/j.nmd.2005.06.019. Epub 2005 Sep 29.

Abstract

We have recently shown that overexpression of ADAM12 results in increased muscle regeneration and significantly reduced pathology in mdx, dystrophin deficient mice. In the present study, we tested the effect of overexpressing ADAM12 in dy(W) laminin-deficient mice. dy mice have a very severe clinical phenotype and would be expected to benefit greatly from enhanced regeneration. We found that dy(W) mice overexpressing ADAM12 indeed have increased muscle regeneration, as evidenced by increased numbers of muscle fibers expressing fetal myosin. However, overexpression of ADAM12 had no significant effect on overall health, as evidenced by body weight, and did not improve muscle pathology.

摘要

我们最近发现,在肌营养不良蛋白缺乏的mdx小鼠中,ADAM12的过表达会导致肌肉再生增加,病理状况显著减轻。在本研究中,我们测试了在dy(W)层粘连蛋白缺陷小鼠中过表达ADAM12的效果。dy小鼠具有非常严重的临床表型,预计会从增强的再生中大大受益。我们发现,过表达ADAM12的dy(W)小鼠确实有肌肉再生增加,表现为表达胎儿肌球蛋白的肌纤维数量增加。然而,ADAM12的过表达对整体健康状况没有显著影响,体重可证明这一点,并且也没有改善肌肉病理状况。

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