The frequency of revertants in mdx mouse genetic models for Duchenne muscular dystrophy

Pediatr Res. 1992 Jul;32(1):128-31. doi: 10.1203/00006450-199207000-00025.


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.

Publication types

  • Research Support, Non-U.S. Gov't
  • Research Support, U.S. Gov't, P.H.S.

MeSH terms

  • Animals
  • Dystrophin / genetics
  • Dystrophin / metabolism
  • Fluorescent Antibody Technique
  • Immunoblotting
  • Mice
  • Mice, Mutant Strains
  • Models, Genetic
  • Muscles / metabolism
  • Muscles / pathology
  • Muscular Dystrophy, Animal / genetics*
  • Muscular Dystrophy, Animal / metabolism
  • Muscular Dystrophy, Animal / pathology
  • Mutation
  • Myocardium / metabolism


  • Dystrophin