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The frequency of revertants in mdx mouse genetic models for Duchenne muscular dystrophy
I Danko1, V Chapman, J A Wolff
1Department of Pediatrics, Waisman Center, University of Wisconsin, Madison 53705.
Pediatric Research
|July 1, 1992
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.