Multivariate evaluation of the functional recovery obtained by the overexpression of utrophin in skeletal muscles of the mdx mouse

Multivariate evaluation of the functional recovery obtained by the overexpression of utrophin in skeletal muscles of the mdx mouse
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DOI:
10.1016/s0960-8966(02)00088-3
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发表时间:
2002-10-01
影响因子:
2.8
通讯作者:
Gillis, JM
Gillis, JM
中科院分区:
医学4区
文献类型:
--
作者:
Gillis, JM

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本文总结了在肌营养不良蛋白缺陷的肌肉中肌营养不良蛋白过表达的mdx小鼠获得的功能恢复的各个方面。这包括四环素控制的utrophin表达的初步结果。它表明,utrophin的过度表达导致主要的功能改善,全长utrophin是更有效的比截短utrophin,缺少中央杆段的一部分。强调了以“恢复评分”的形式呈现任何治疗所获得的改善的一般方式。肌营养不良蛋白的替代的定量方面进行了讨论。(C)2002 Elsevier Science B.V.保留所有权利。
This paper summarizes the various aspects of functional recovery obtained in dystrophin-deficient muscles of the mdx mice where utrophin was overexpressed. This includes preliminary results on tetracycline-controlled expression of utrophin. It is shown that overexpression of utrophin leads to major functional improvements and that full-length utrophin is more efficient than truncated utrophin, missing a part of the central rod-segment. A generalized way of presenting improvements obtained by any treatment in the form of a 'recovery score' is emphasized. The quantitative aspect of the replacement of dystrophin by utrophin is discussed. (C) 2002 Elsevier Science B.V. All rights reserved.