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Neuromuscul Disord. 2002 Oct;12 Suppl 1:S90-4.

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

Author information

1
Département de Physiologie, Université Catholique de Louvain, UCL 5540, Avenue Hippocrate 55, 1200 Brussels, Belgium. gillis@fymu.ucl.ac.be

Abstract

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.

PMID:
12206802
DOI:
10.1016/s0960-8966(02)00088-3
[Indexed for MEDLINE]

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