Fetal muscle-derived cells can repair dystrophic muscles in mdx mice
- CNRS UMR 6204, Faculte des Sciences et des Techniques, 44322 Nantes Cedex 3 (France)
We have previously reported that CD34{sup +} cells purified from mouse fetal muscles can differentiate into skeletal muscle in vitro and in vivo when injected into muscle tissue of dystrophic mdx mice. In this study, we investigate the ability of such donor cells to restore dystrophin expression, and to improve the functional muscle capacity of the extensor digitorum longus muscle (EDL) of mdx mice. For this purpose green fluorescent-positive fetal GFP{sup +}/CD34{sup +} cells or desmin{sup +}/{sup -}LacZ/CD34{sup +} cells were transplanted into irradiated or non-irradiated mdx EDL muscle. Donor fetal muscle-derived cells predominantly fused with existing fibers. Indeed more than 50% of the myofibers of the host EDL contained donor nuclei delivering dystrophin along 80-90% of the length of their sarcolemma. The presence of significant amounts of dystrophin (about 60-70% of that found in a control wild-type mouse muscle) was confirmed by Western blot analyses. Dystrophin expression also outcompeted that of utrophin, as revealed by a spatial shift in the distribution of utrophin. At 1 month post-transplant, the recipient muscle appeared to have greater resistance to fatigue than control mdx EDL muscle during repeated maximal contractions.
- OSTI ID:
- 20972131
- Journal Information:
- Experimental Cell Research, Vol. 313, Issue 5; Other Information: DOI: 10.1016/j.yexcr.2006.12.021; PII: S0014-4827(06)00526-X; Copyright (c) 2007 Elsevier Science B.V., Amsterdam, The Netherlands, All rights reserved; Country of input: International Atomic Energy Agency (IAEA); ISSN 0014-4827
- Country of Publication:
- United States
- Language:
- English
Similar Records
Truncated dystrophin ameliorates the dystrophic phenotype of mdx mice by reducing sarcolipin-mediated SERCA inhibition
Effects of use and disuse on growing skeletal muscle