Journal
NEUROMUSCULAR DISORDERS
Volume 12, Issue -, Pages S90-S94Publisher
PERGAMON-ELSEVIER SCIENCE LTD
DOI: 10.1016/S0960-8966(02)00088-3
Keywords
utrophin; dystrophin; skeletal muscle; mdx mouse
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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.
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