Transplantation of myoblasts from a transgenic mouse overexpressing dystrophin prduced only a relatively small increase of dystrophin-positive membrane

Muscle & Nerve
I KinoshitaJacques P Tremblay

Abstract

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 beta-gal transgene (Tg-MDA/beta-gal) and myoblasts from beta-gal transgenic mice containing one normal dystrophin gene (normal/beta-gal) were also transplanted into mdx mouse muscles. Four weeks after transplantation, the fiber length positive for dystrophin (nuclear domain) was shorter (439 +/- 326 microm) than the beta-gal nuclear domain (1466 +/- 713 microm) of the same fiber when normal/beta-gal myoblasts were transplanted, but increased (1302 +/- 487 microm) when Tg-MDA/beta-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.

References

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Citations

Jun 18, 2002·Journal of Cellular and Molecular Medicine·G M SmytheM D Grounds
Jan 6, 2011·Expert Opinion on Biological Therapy·Daniel Skuk, Jacques P Tremblay
Dec 2, 2004·Expert Opinion on Biological Therapy·Daniel Skuk
Jan 27, 2016·Molecular Therapy. Nucleic Acids·Jean-Paul Iyombe-EngembeJacques P Tremblay
Sep 29, 2011·The Journal of Gene Medicine·Joel RousseauJacques P Tremblay
May 15, 2015·Nature Communications·Alexandra C KeefeGabrielle Kardon
Jan 11, 2001·Muscle & Nerve·J Kong, J E Anderson
Dec 11, 1999·Developmental Dynamics : an Official Publication of the American Association of Anatomists·K BlaveriJ E Morgan
Aug 10, 2000·Molecular Therapy : the Journal of the American Society of Gene Therapy·G M SmytheM D Grounds
Aug 20, 2010·Cell Transplantation·Anabel S de la Garza-RodeaShoshan Knaän-Shanzer
Apr 16, 2010·Gene Therapy·P ChapdelaineJ P Tremblay
Aug 28, 2001·Journal of Biotechnology·P BoudreaultA Garnier

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