重症肌无力患者骨骼肌肌肉蛋白成分分析

　　【摘要】　目的　探讨重症肌无力（MG）肌肉中与收缩有关的肌球蛋白成分是否减少。方法　 用Gubstraub溶液(myosin 提取液) 按常量（匀浆）和微量（浸出）方式提取10名正常人及17例MG患者肌肉的蛋白成分，用双盲法分别以常量及微量聚丙烯酰胺凝胶电泳(SDS-PAGE)方法分析肌肉蛋白成分, 用双向电泳分析有差异的谱带。结果　常量SDS-PAGE电泳图谱分析显示,相对分子质量约25 000的蛋白谱带密度为4.14±1.33，MG患者肌肉为2.02±1.08，差异有非常显著意义（P＜0.001）；微量分析获得正常肌肉该蛋白谱带的密度为4.26±2.58，MG患者肌肉为1.34±0.79，差异亦有非常显著意义（P＜0.001）；双向电泳显示，有差异的25 000蛋白略偏碱性，并至少由2个等电点相近的蛋白成分组成。用部分纯化的肌球蛋白鉴定显示，25 000蛋白与肌球蛋白轻链无关。结论　MG患者肌肉中25 000蛋白水平明显低于正常人肌肉，提示该蛋白可能与MG发病或发展有关。

Analysis of protein components of skeletal muscle from the patients with myasthenia gravis

REN Huimin, ZHOU Zhigang, CHEN Xiangjun, et al.

　　nstitute of Neurology, Medical Center of Fudan University, Shanghai 200040, China

　　【Abstract】　Objective　To explore whether the components of protein associated with muscle contraction appear decreased in the muscles of myasthenia gravis (MG).Methods　The proteins were extracted from 10 normal cases and 17 cases of MG skeletal muscles with Gubstraub solution, and the components of the protein were analyzed by SDS-PAGE in common and micro methods in double blind. Composition of the differential protein band was observed by two-dimensional electrophoresis.Results　SDS-PAGE patterns showed that the density of the protein band with a mass of about 25 kD in MG muscles was much lower than that in normal muscles. The value of density for 25 kD protein band in common analysis was 4.14±1.33 and 2.02±1.08 in the normal and MG muscles, respectively (P＜0.001), while in micro analysis the density of the protein band was 4.26±2.58 in normal and 1.34±0.79 in MG muscles, respectively (P＜0.001).The pattern of two-dimensional electrophoresis demonstrated that the differential 25 kD protein band was composed of more than two components appearing in the adjacent isoelectric point on the alkaline side of the gel, and they were proved to be not related to the components of myosin light chains. Conclusion　It was suggested that pathogenically the development of MG should be associa

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