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Publication : Contractile properties of diaphragm muscle segments from old mdx and old transgenic mdx mice.

First Author  Lynch GS Year  1997
Journal  Am J Physiol Volume  272
Issue  6 Pt 1 Pages  C2063-8
PubMed ID  9227435 Mgi Jnum  J:113048
Mgi Id  MGI:3664381 Doi  10.1152/ajpcell.1997.272.6.C2063
Citation  Lynch GS, et al. (1997) Contractile properties of diaphragm muscle segments from old mdx and old transgenic mdx mice. Am J Physiol 272(6 Pt 1):C2063-8
abstractText  Diaphragm muscles of young (4- to 6-mo-old) mdx mice show severe fiber necrosis and have normalized forces and powers 60 and 46% of the values for control C57BL/10 mice. In contrast, microinjection of mdx mouse embryos with a truncated dystrophin minigene has produced young transgenic mdx (tg-mdx) mice with a level of dystrophin expression and structural and functional properties of diaphragm muscle strips measured in vitro not different from those of control mice. Whether dystrophin expression and functional corrections persist for the life span of these animals is not know. We tested the null hypothesis that, in old (24 mo) tg-mdx mice, dystrophin expression is adequate and diaphragm muscle strips have forces and powers not different from values for diaphragm muscle strips from young tg-mdx mice or control mice. Compared with control values, diaphragm muscle strips from old mdx mice had normalized forces and powers of 48 and 31%, respectively. Expression of dystrophin persisted in diaphragm muscles of old tg-mdx mice, and functional properties were not different from diaphragm muscles of young tg-mdx or young or old control mice. These results suggest that, with a transgenic animal approach, dystrophin expression and functional corrections persist for the life span of the animals.
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