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

M Hino, N Hamada, Y Tajika, T Funayama… - Cell structure and …, 2009 - jstage.jst.go.jp
M Hino, N Hamada, Y Tajika, T Funayama, Y Morimura, T Sakashita, Y Yokota, K Fukamoto…
Cell structure and function, 2009jstage.jst.go.jp
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 …
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.
jstage.jst.go.jp