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Dystrophin distribution in heterozygote mdx mice
Author(s) -
Watkins Simon C.,
Hoffman P.,
Slayter Henry S.,
Kunkel Louis M.
Publication year - 1989
Publication title -
muscle and nerve
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.025
H-Index - 145
eISSN - 1097-4598
pISSN - 0148-639X
DOI - 10.1002/mus.880121013
Subject(s) - dystrophin , immunostaining , mdx mouse , utrophin , heterozygote advantage , duchenne muscular dystrophy , biology , muscular dystrophy , pathology , microbiology and biotechnology , endocrinology , immunohistochemistry , medicine , gene , immunology , genetics , genotype
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.