THE FREQUENCY OF REVERTANTS IN MDX MOUSE GENETIC MODELS FOR DUCHENNE MUSCULAR-DYSTROPHY

被引:123
作者
DANKO, I
CHAPMAN, V
WOLFF, JA
机构
[1] UNIV WISCONSIN,WAISMAN CTR,DEPT PEDIAT,1500 HIGHLAND AVE,MADISON,WI 53705
[2] NEW YORK STATE DEPT HLTH,ROSWELL PK MEM INST,DEPT MOLEC & CELLULAR BIOL,BUFFALO,NY 14263
[3] UNIV WISCONSIN,WAISMAN CTR,DEPT MED GENET,MADISON,WI 53705
关键词
D O I
10.1203/00006450-199207000-00025
中图分类号
R72 [儿科学];
学科分类号
100202 ;
摘要
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.
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页码:128 / 131
页数:4
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