Functional protection of dystrophic mouse (mdx) muscles after adenovirus-mediated transfer of a dystrophin minigene
(1996) Proceedings of the National Academy of Sciences of the United States of America — Vol. 93, n° 8, p. 3570-3574 (1996)
Files
8622977.pdf
Open Access - Adobe PDF
- 1.37 MB
Details
- Authors
- Abstract
- Fast skeletal muscles of mdx (X chromosome-linked muscular dystrophy) mice were injected after birth with a recombinant adenovirus containing a minidys- trophin gene, a 6.3-kbp cDNA coding for the N- and C-terminal ends of dystrophin. Adult muscles were challenged by forced lengthening during tetanic contractions. Stretch-induced mechanical and histological damages were much reduced in injected muscles, in direct proportion of the Miniber of fibers expressing minidystrophin. Damaged fibers were preferentially found among minidystrophin-negative regions. Minidystrostrophin confers an important functional and structural protection of limb muscles against high mechanical stress, even after a partial somatic gene transfer.
- Affiliations
Citations
Deconinck, N., Ragot, T., Maréchal, G., Perricaudet, M., & Gillis, J.-M. (1996). Functional protection of dystrophic mouse (mdx) muscles after adenovirus-mediated transfer of a dystrophin minigene. Proceedings of the National Academy of Sciences of the United States of America, 93(8), 3570-3574. https://doi.org/10.1073/pnas.93.8.3570 (Original work published 1996)