Gillis Jean-Marie
Département de Physiologie, Université Catholique de Louvain, UCL 5540, Avenue Hippocrate 55, 1200 Brussels, Belgium.
Neuromuscul Disord. 2002 Oct;12 Suppl 1:S90-4. doi: 10.1016/s0960-8966(02)00088-3.
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.