title: Insufficient membrane fusion in dysferlin-deficient muscle fibers after heavy-ion irradiation.
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.
authors.affiliation: Department of Anatomy, Division of Bioregulatory Medicine, Gunma University Graduate School of Medicine, Japan.
authors.forename: Mizuki
authors.initials: M
authors.lastname: Hino
authors.forename: Nobuyuki
authors.initials: N
authors.lastname: Hamada
authors.forename: Yuki
authors.initials: Y
authors.lastname: Tajika
authors.forename: Tomoo
authors.initials: T
authors.lastname: Funayama
authors.forename: Yoshihiro
authors.initials: Y
authors.lastname: Morimura
authors.forename: Tetsuya
authors.initials: T
authors.lastname: Sakashita
authors.forename: Yuichiro
authors.initials: Y
authors.lastname: Yokota
authors.forename: Kana
authors.initials: K
authors.lastname: Fukamoto
authors.forename: Yasuhiko
authors.initials: Y
authors.lastname: Kobayashi
authors.forename: Hiroshi
authors.initials: H
authors.lastname: Yorifuji
mesh_terms: D000818:Animals; D001343:Autophagy; D002462:Cell Membrane / Q000378:metabolism / Q000648:ultrastructure; D002478:Cells, Cultured; D000073939:Dysferlin; D005260:Female; D006801:Humans; D008561:Membrane Fusion / Q000502:physiology*; D008565:Membrane Proteins / Q000235:genetics / Q000378:metabolism*; D051379:Mice; D018345:Mice, Knockout; D018485:Muscle Fibers, Skeletal / Q000473:pathology / Q000528:radiation effects* / Q000648:ultrastructure*; D011839:Radiation, Ionizing
chemical_list: C497505:Dysf protein, mouse; D000073939:Dysferlin; D008565:Membrane Proteins