Insufficient membrane fusion in dysferlin-deficient muscle fibers after heavy-ion irradiation

Mizuki Hino, Nobuyuki Hamada, Yuki Tajika, Tomoo Funayama, Yoshihiro Morimura, Tetsuya Sakashita, Yuichiro Yokota, Kana Fukamoto, Yasuhiko Kobayashi, Hiroshi Yorifuji

Research output: Contribution to journalArticlepeer-review

Abstract

Recently, SJL/J mice have been used as an animal model in studies of dysferlinopathy, a spectrum of muscle diseases caused by defects in dysferlin protein. In this study we irradiated muscle fibers isolated from skeletal muscle of SJL/J mice with heavy-ion microbeam, and the ultrastructural changes were observed by electron microscopy. The plasma membrane of heavy-ion beam irradiated areas showed irregular protrusions and invaginations. Disruption of sarcomeric structures and the enhancement of autophagy were also observed. In addition, many vesicles of varying size and shape were seen to be accumulated just beneath the plasma membrane. This finding further supports the recent hypothesis that dysferlin functions as a membrane fusion protein in the wound healing system of plasma membrane, and that the defect in dysferlin causes insufficient membrane fusion resulting in accumulation of vesicles.

Original languageEnglish
Pages (from-to)11-15
Number of pages5
JournalCell structure and function
Volume34
Issue number1
DOIs
Publication statusPublished - 2009
Externally publishedYes

Keywords

  • Dysferlin
  • Electron microscopy
  • Heavy-ion microbeam
  • Membrane repair
  • Vesicle accumulation

ASJC Scopus subject areas

  • Physiology
  • Molecular Biology
  • Cell Biology

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