Dystrophin expression improves myofiber survival in mdx muscle following intramuscular plasmid DNA injection.
Expression of Becker-like and full-length human dystrophins was stable for at least 6 months in mdx mouse muscle following intramuscular plasmid DNA injection. Intramuscular injection of a single plasmid DNA encoding both luciferase and dystrophin resulted in stable luciferase expression for at least 2 months in mdx muscle, whereas injection of plasmid DNA encoding only luciferase did not result in stable luciferase expression. These results suggest that expression of either full-length or Becker-like dystrophins protects mdx mouse myofibers from degeneration.
Danko
I
I
Department of Pediatrics, Waisman Center, University of Wisconsin-Madison 53705.
Fritz
J D
JD
Latendresse
J S
JS
Herweijer
H
H
Schultz
E
E
Wolff
J A
JA
D000818:Animals; D002470:Cell Survival; D004247:DNA / Q000008:administration & dosage / Q000235:genetics / Q000378:metabolism*; D016189:Dystrophin / Q000096:biosynthesis* / Q000378:metabolism; D006801:Humans; D007273:Injections, Intramuscular; D008156:Luciferases / Q000032:analysis / Q000096:biosynthesis; D051379:Mice; D008807:Mice, Inbred BALB C; D008810:Mice, Inbred C57BL; D008813:Mice, Inbred ICR; D008818:Mice, Neurologic Mutants; D009132:Muscles / Q000378:metabolism / Q000473:pathology*; D009137:Muscular Dystrophy, Animal / Q000378:metabolism / Q000473:pathology* / Q000628:therapy; D010957:Plasmids / Q000008:administration & dosage*; D011993:Recombinant Fusion Proteins / Q000096:biosynthesis; D001616:beta-Galactosidase / Q000032:analysis / Q000096:biosynthesis
D016189:Dystrophin; D011993:Recombinant Fusion Proteins; D004247:DNA; D008156:Luciferases; D001616:beta-Galactosidase