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Transplantation of myoblasts from a transgenic mouse overexpressing dystrophin produced only a relatively small increase of dystrophin‐positive membrane
Author(s) -
Kinoshita I.,
Vilquin J.T.,
Asselin I.,
Chamberlain J.,
Tremblay J.P.
Publication year - 1998
Publication title -
muscle and nerve
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.025
H-Index - 145
eISSN - 1097-4598
pISSN - 0148-639X
DOI - 10.1002/(sici)1097-4598(199801)21:1<91::aid-mus12>3.0.co;2-3
Subject(s) - dystrophin , transplantation , transgene , genetically modified mouse , myocyte , duchenne muscular dystrophy , medicine , myopathy , biology , microbiology and biotechnology , pathology , genetics , gene
Myoblast cultures from normal and Tg‐MDA (transgenic mouse overexpressing dystrophin 50‐fold) mice were transplanted into dystrophin‐deficient mdx mouse muscles. Four weeks after transplantation, dystrophin‐positive fibers were observed four times more frequently in cross sections of muscles injected with Tg‐MDA. Myoblasts from Tg‐MDA mice also expressing the β‐gal transgene (Tg‐MDA/β‐gal) and myoblasts from β‐gal transgenic mice containing one normal dystrophin gene (normal/β‐gal) were also transplanted into mdx mouse muscles. Four weeks after transplantation, the fiber length positive for dystrophin (nuclear domain) was shorter (439 ± 326 μm) than the β‐gal nuclear domain (1466 ± 713 μm) of the same fiber when normal/β‐gal myoblasts were transplanted, but increased (1302 ± 487 μm) when Tg‐MDA/β‐gal myoblasts were used. These experiments show that despite the presence in Tg‐MDA myoblasts of constructions which lead in vivo in transgenic mice to an overexpression of dystrophin 50‐fold, the membrane area over which dystrophin was expressed was increased only threefold. This observation is also expected for vector‐mediated gene therapy. © 1998 John Wiley & Sons, Inc. Muscle Nerve, 21: 91–103, 1998.