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本文对2例线粒体肌病患者腓肠肌活检组织进行光镜和电镜超微病理观察,发现病变肌纤维超微结构变化丰富,尤其1例患者线粒体内类结晶包涵体形态独特,且存在脂滴沉积、糖原颗粒聚集。通过分析线粒体肌病电镜图像变化的特征,探讨该病的病因和可能的发病机制,即线粒体内不同形态的类结晶包涵体可能代表了不同种蛋白质的堆积 ,或是同一种蛋白质异常合成的不同时期的表现;脂滴沉积和糖原颗粒的异常增多,是由于线粒体功能障碍所致。
In this paper, two cases of mitral myopathy patients gastrocnemius muscle biopsies by light microscopy and electron microscopy pathological findings found that the pathological changes of muscle fiber ultrastructure rich, in particular, one case of mitochondrial endometrial crystal inclusions unique morphology, and the presence of lipid droplets deposition, sugar The original particle aggregation. By analyzing the characteristics of mitochondrial EMG changes, we explore the etiology and possible pathogenesis of mitochondrial myofibrils, that is, the different types of crystal inclusion bodies in mitochondria may represent the accumulation of different kinds of proteins or the different synthesis of the same kind of proteins Period of performance; lipid droplet deposition and abnormal increase in glycogen particles, due to mitochondrial dysfunction.