Dystrophin distribution in heterozygote MDX mice.
The distribution of dystrophin in myofibers from normal, mdx hemizygous, and mdx heterozygous mice was studied at various times in development. While normal mice exhibit dystrophin immunostaining around the entire fiber periphery regardless of age, mdx hemizygous mice exhibit no staining (0-35 days). In contrast, young (10 day) heterozygous mdx mice showed neighboring dystrophin-negative and dystrophin-positive fibers as well as fibers with a discontinuous or patchy dystrophin labelling. Older heterozygotes displayed very few negative fibers, with most fibers exhibiting apparently complete dystrophin immunostaining. This, coupled with the absence of muscle fiber degeneration at any age point, and the apparently normal levels of dystrophin in older heterozygous mice, indicates that myonuclei containing the dystrophin gene can compensate for myonuclei which do not contain the dystrophin gene within the same myofiber.
Watkins
S C
SC
Laboratory of Structural Molecular Biology, Dana-Farber Cancer Institute, Boston, MA 02115.
Hoffman
E P
EP
Slayter
H S
HS
Kunkel
L M
LM
D000818:Animals; D016189:Dystrophin; D005455:Fluorescent Antibody Technique; D006579:Heterozygote; D051379:Mice; D008810:Mice, Inbred C57BL; D009124:Muscle Proteins / Q000032:analysis*; D009132:Muscles / Q000032:analysis; D009137:Muscular Dystrophy, Animal / Q000378:metabolism*; D013194:Staining and Labeling
D016189:Dystrophin; D009124:Muscle Proteins