Insufficient membrane fusion in dysferlin-deficient muscle fibers after heavy-ion irradiation
| Autor: | Nobuyuki Hamada, Yasuhiko Kobayashi, Mizuki Hino, Yoshihiro Morimura, Kana Fukamoto, Hiroshi Yorifuji, Tomoo Funayama, Yuki Tajika, Tetsuya Sakashita, Yuichiro Yokota |
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| Rok vydání: | 2009 |
| Předmět: |
Dysferlinopathy
Physiology Muscle Fibers Skeletal Membrane Fusion Dysferlin Mice Radiation Ionizing medicine Autophagy Animals Humans Molecular Biology Cells Cultured Mice Knockout biology Membrane fusion protein Chemistry Vesicle Cell Membrane Skeletal muscle Lipid bilayer fusion Membrane Proteins Cell Biology General Medicine medicine.disease medicine.anatomical_structure Biochemistry biology.protein Biophysics Female Wound healing |
| Zdroj: | Cell structure and function. 34(1) |
| ISSN: | 1347-3700 |
| Popis: | 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. |
| Databáze: | OpenAIRE |
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