Dystrophin distribution in heterozygote MDX mice

Abstract

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.