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Observations of muscle plasma membrane undercoats in Duchenne and fukuyama muscula dystrophies |
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| Authors: | Makoto Murahashi Yoshihiro Wakayama Toshiyuki Kumagai Takuya Kobayashi Sumimasa Yamashita Nobuko Misugi Shota Miyake Seiji Shibuya Takahiro Jimi and Hiroaki Oniki |
| Institution: | (1) Division of Neurology, Department of Medicine, Showa University Fujigaoka Hospital, 1-30, Fujigaoka, Aoba-ku, 227 Yokohama, Japan;(2) Electron Microscopic Laboratory, Showa University Fujigaoka Hospital, 1-30, Fujigaoka, Aoba-ku, 227 Yokohama, Japan;(3) Division of Pediatric Neurology, Central Hospital, Aichi Prefectural Colony, 713-8, Kamiya-cho, 480-03 Kasugai, Japan;(4) Department of Pediatrics, Yokohama City University School of Medicine, 3-46, Urafune-cho, Minami-ku, 231 Yokohama, Japan;(5) Department of Neurology, Kanagawa Children's Medical Center, 2-138-4, Mutsugawa, Minami-ku, 232 Yokohama, Japan;(6) Department of Orthopeics, Kanagawa Children's Medical Center, 2-138-4, Mutsugawa, Minami-ku, 232 Yokohama, Japan |
| Abstract: | Muscle plasma membrane undercoats were investigated by conventional electron microscopy in both Duchenne muscular dystrophy (DMD) and Fukuyama congenital muscular dystrophy (FCMD). The densities of the plasma membrane undercoats were rarefied in the parts of the plasma membranes overlying the degenerating focus in both DMD and FCMD myofibers. The degree of rarefaction tended to be parallel to the degree of degeneration in the myofibers. It was hard to distinguish the undercoat densities of normal-looking myofibers of DMD and FCMD muscles from those of control myofibers from histochemically-normal muscles. On the other hand, the undercoats of regenerating myofibers in DMD and FCMD muscles were denser than normal. |
| Keywords: | Duchenne muscular dystrophy Fukuyama congenital muscular dystrophy Electron microscopy Myofiber degeneration and regeneration Muscle plasma membrane undercoat |
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