Watkins S C, Cullen M J
Core Electron Microscopy Laboratory, Dana Farber Cancer Institute, Boston, MA.
J Neurol Sci. 1987 Dec;82(1-3):181-92. doi: 10.1016/0022-510x(87)90017-7.
Quantitative and qualitative ultrastructural analyses were made of selected small muscle fibres from muscle biopsies from 23 patients with Duchenne muscular dystrophy (DMD) and 10 with polymyositis. It is argued that the fibres selected were regenerating. The myofibrillar content of the fibres was similar in both samples. In both there was considerable misalignment of the myofibrils but their orientation tended to improve as the content increased. The mitochondrial content was similar in the two samples but there were more ultrastructural abnormalities in the DMD population. The concentration of glycogen was elevated and the sarcoplasmic reticulum (SR) was much more commonly dilated in the DMD patients. Dilated SR is the prenecrotic change seen most frequently in mature muscle fibres in DMD and seems to be the first ultrastructural manifestation of the disease process. Its occurrence in the regenerating fibres suggest that the primary genetic lesion is being recapitulated in successive generations of fibres.
对23例杜氏肌营养不良症（DMD）患者和10例多发性肌炎患者肌肉活检所选取的小肌纤维进行了超微结构的定量和定性分析。据认为所选取的纤维正在再生。两个样本中纤维的肌原纤维含量相似。两者的肌原纤维均有相当程度的排列紊乱，但随着含量增加其排列倾向于改善。两个样本中的线粒体含量相似，但DMD患者群体中存在更多超微结构异常。糖原浓度升高，DMD患者的肌浆网（SR）扩张更为常见。扩张的SR是DMD成熟肌纤维中最常见的坏死前变化，似乎是疾病过程的首个超微结构表现。其在再生纤维中的出现表明原发性基因病变在纤维的连续几代中得以重现。
