title: The frequency of revertants in mdx mouse genetic models for Duchenne muscular dystrophy.
abstract: The mdx mouse has been used for the development of cellular and gene therapies for Duchenne muscular dystrophy. The relatively frequent occurrence of dystrophin-positive muscle cells called revertants has hampered these efforts by interfering with data interpretation. The mdx4cv and mdx5cv dystrophin mouse mutants have approximately 10-fold fewer revertants than the mdx mutant at both 2 and 6 mo. The mdx3cv dystrophin mouse mutant may be a useful model for some types of human dystrophin deficiencies in which the levels of dystrophin are low but not completely absent.
authors.affiliation: Department of Pediatrics, Waisman Center, University of Wisconsin, Madison 53705.
authors.forename: I
authors.initials: I
authors.lastname: Danko
authors.forename: V
authors.initials: V
authors.lastname: Chapman
authors.forename: J A
authors.initials: JA
authors.lastname: Wolff
mesh_terms: D000818:Animals; D016189:Dystrophin / Q000235:genetics / Q000378:metabolism; D005455:Fluorescent Antibody Technique; D015151:Immunoblotting; D051379:Mice; D008817:Mice, Mutant Strains; D008957:Models, Genetic; D009132:Muscles / Q000378:metabolism / Q000473:pathology; D009137:Muscular Dystrophy, Animal / Q000235:genetics* / Q000378:metabolism / Q000473:pathology; D009154:Mutation; D009206:Myocardium / Q000378:metabolism
chemical_list: D016189:Dystrophin