Deconinck, N.
Ragot, T
Maréchal, Georges
[UCL]
Perricaudet, M.
Gillis, Jean-Marie
[UCL]
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.
Bibliographic reference |
Deconinck, N. ; Ragot, T ; Maréchal, Georges ; Perricaudet, M. ; Gillis, Jean-Marie. Functional protection of dystrophic mouse (mdx) muscles after adenovirus-mediated transfer of a dystrophin minigene.. In: Proceedings of the National Academy of Sciences of the United States of America, Vol. 93, no. 8, p. 3570-4 (1996) |
Permanent URL |
http://hdl.handle.net/2078.1/12169 |