Insufficient Membrane Fusion in Dysferlin-Deficient Muscle Fibers after Heavy-Ion Irradiation
Insufficient Membrane Fusion in Dysferlin-Deficient Muscle Fibers after Heavy-Ion Irradiation
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DOI：
10.1247/csf.08033
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发表时间：
2009-01-01
影响因子：
1.5
通讯作者：
Yorifuji, Hiroshi
中科院分区：
文献类型：
--
作者：
Hino, Mizuki;Hamada, Nobuyuki;Yorifuji, Hiroshi
最近，SJL/J小鼠已被用作研究dysferlin病的动物模型，dysferlin病是由dysferlin蛋白缺陷引起的一系列肌肉疾病。本研究用重离子微束辐照SJL/J小鼠骨骼肌，观察其肌纤维超微结构的变化。重离子束辐照区的质膜出现不规则的突起和内陷。还观察到肌节结构的破坏和自噬的增强。此外，许多不同大小和形状的囊泡被认为是积累在质膜下。这一发现进一步支持了最近的假设，即dysferlin在质膜的伤口愈合系统中作为膜融合蛋白发挥作用，并且dysferlin的缺陷导致膜融合不足，从而导致囊泡的积累。
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.