线粒体肌病超微结构特征研究

目的：结合线粒体肌病超微结构变化的特征，探讨该病的病因和可能的发病机制。方法：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。结果：发现病变肌纤维超微结构变化丰富，线粒体内类结晶包涵体形态独特，肌纤维间存在脂滴沉积，糖原颗粒聚集和异形吞噬体。结论：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，是继发于线粒体功能的障碍所致，进而出现形吞噬体的自我保护性反应。

A study on ultrastructural changes of mitochondrial myopathy

Objective: To inquire into the pathological significance of ultrastructural changes and the possible cause and pathogenesis in patients with mitochondrial myopathy. Methods: The specimens of muscle biopsy taken from 2 patients were studied with light microscopy and transmission electron microscopy(TEM). Results: TEM shows that the myofibers possess an excessive number of mitochondria, mainly accumulated beneath sarcolemma. Many mitochondria are enlarged and present structural abnormalities such as paracrystalline inclusions of various shapes. Affected fibers also contain increased number of lipid droplets and glycogen granules and phagolysosome. Conclusion: The various paracrystalline inclusions in mitochondrial may represent the accumulation of different kinds of proteins or different processes of protein synthesis. It further results in accumulation of lipid droplets, glycogen granules and appearance of phagolysosome.