Uchino M, Araki S, Yoshida O, Uekawa K
Neurology. 1985 Sep;35(9):1364-7. doi: 10.1212/wnl.35.9.1364.
We studied the structural proteins of the "opaque fibers" (OFs) in muscle biopsies from 12 patients, aged 4 to 24 years, with Duchenne muscular dystrophy. The electrophoretic patterns of myofibrillar proteins indicated three groups of opaque fibers: the first (OF a) had an almost normal electrophoretogram, the second (OF b1) showed a clear decrease of 55,000 dalton protein (desmin), and the third (OF b2) exhibited a great reduction in alpha-actinin and desmin. The data suggest that some OFs are pathologic fibers in the degenerative process of Duchenne dystrophy.
我们研究了12名年龄在4至24岁之间的杜氏肌营养不良症患者肌肉活检中“不透明纤维”(OFs)的结构蛋白。肌原纤维蛋白的电泳图谱显示有三组不透明纤维:第一组(OF a)的电泳图几乎正常,第二组(OF b1)显示55,000道尔顿蛋白(结蛋白)明显减少,第三组(OF b2)的α-辅肌动蛋白和结蛋白大幅减少。数据表明,一些不透明纤维是杜氏肌营养不良症退行性过程中的病理性纤维。