Insufficient Membrane Fusion in Dysferlin-Deficient Muscle Fibers after Heavy-Ion Irradiation

Cell Structure and Function

Mizuki Hino²  Yoshihiro Morimura²  Tetsuya Sakashita¹  Tomoo Funayama¹  Nobuyuki Hamada³  Yuki Tajika²  Yasuhiko Kobayashi³  Kana Fukamoto¹  Hiroshi Yorifuji²  Yuichiro Yokota¹ 

[1] Microbeam Radiation Biology Group, Radiation-applied Biology Division, Quantum Beam Science Directorate, Japan Atomic Energy Agency;Department of Anatomy, Division of Bioregulatory Medicine, Gunma University Graduate School of Medicine;The 21st Century Center of Excellence Program for Biomedical Research Using Accelerator Technology

关键词: heavy-ion microbeam;    dysferlin;    electron microscopy;    vesicle accumulation;    membrane repair;   

DOI  :  10.1247/csf.08033

学科分类：分子生物学，细胞生物学和基因

来源: Japan Society for Cell Biology

【 摘 要 】

References(24)Cited-By(9)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.

【 授权许可】

Unknown