@article{oai:sapmed.repo.nii.ac.jp:00014656,
 author = {渡辺, 幸恵 and 舘, 延忠},
 issue = {6},
 journal = {札幌医学雑誌 = The Sapporo medical journal, The Sapporo medical journal},
 month = {Dec},
 note = {The expression of dystrophin and dystrophin-related protein （DRP） in biopsied and cultured muscles from eight Duchenne muscular dystrophy （DMD） patients, four DMD carriers, seven Becker muscular dystrophy （BMD） patients, two dermatomyositis patients and ten normal controls were studied by immunocytochemistry and Western blot analysis. In muscle biopsies, DRP was located on the sarcolemma of dystrophin deficient muscle fibers from patients with DMD, BMD and DMD carriers and regenerating muscle fibers from patients with dermatomyositis. Successive sections of muscles from patients with DMD, DMD carrier, BMD, dermatomyositis, and controls were stained with anti-dystrophin antibodies and anti-DRP antibodies. DRP expression on dystrophin deficient fibers did not completely compensate dystrophin. In muscle cultures, dystrophin was not demonstrable in undifferentiated myoblasts from patients with DMD and controls. After myoblast fusion from control muscles, dystrophin appeared in the cytoplasm of myotubes as discrete round packages in perinuclear resions, and along the surface of more mature myotubes. There was no staining in myotubes from DMD patients. DRP was expressed in the cytoplasm of myoblasts and myotubes from control patients and patients with DMD. Thus far, these results indicate that DRP was expressed by activation of satellite cell irrespective of the cause of muscle damage. Some DMD patients had rare staining dystrophin-positive fibres, termed "revertant fibers". Immunocytochemistry was performed on serial sections from deleted and non-deleted patients by using a panel of anti-dystrophin antibodies. In patients with deletions, revertant fibers did not stain with antibody raised to polypeptide sequences within the deletion. Based on analysis of dystrophin protein in revertant fibers, the most likely mechanism giving rise to revertant fibers appers to be a second site in-frame deletion.},
 pages = {227--237},
 title = {生検筋と培養筋におけるDystrophinとDystrophin related proteinの発現について : Duchenne型筋ジストロフィーを中心に},
 volume = {64},
 year = {1995}
}