Doriguzzi C, Palmucci L, Pollo B, Mongini T, Maniscalco M, Chiadò-Piat L, Schiffer D
Centro per le Malattie Neuromuscolari Paolo Peirolo, Clinica Neurologica II. Università di Torino, Italy.
Acta Neuropathol. 1990;81(1):25-9. doi: 10.1007/BF00662634.
Cytochrome c oxidase (CCO) has been histochemically studied in 250 muscle biopsies from patients with different neuromuscular diseases. The results were compared with those obtained on serial sections stained with Gomori's trichrome and with the methods for NADH tetrazolium reductase, succinate dehydrogenase and lactate dehydrogenase. In 58 selected cases serial sections were also stained with a method demonstrating coenzyme Q (CoQ) activity. Demonstration of structural alterations was as good with CCO as with the methods for other oxidative enzymes: particularly evident were alterations of the distribution of mitochondria, such as core areas in central core and multiminicore diseases. Unstained fibers were observed in mitochondrial myopathies, in Becker, Emery-Dreifuss, limb-girdle, facio-scapulo-humeral muscular dystrophies, muscle infarction, polymyositis, motor neuron diseases and neuropathies. The histochemical method for CoQ showed only low specificity, since partial staining was also present in areas devoid of mitochondria, such as cores. CoQ deficiency was not observed in any of the 19 mitochondrial myopathies examined.
细胞色素c氧化酶（CCO）已在250例患有不同神经肌肉疾病患者的肌肉活检组织中进行了组织化学研究。将结果与用Gomori三色染色法的连续切片以及烟酰胺腺嘌呤二核苷酸四唑还原酶、琥珀酸脱氢酶和乳酸脱氢酶检测方法所获得的结果进行了比较。在58例选定病例中，连续切片还用一种显示辅酶Q（CoQ）活性的方法进行了染色。CCO对结构改变的显示效果与其他氧化酶检测方法相同：线粒体分布的改变尤为明显，如中央轴空病和多微小轴空病中的核心区域。在线粒体肌病、贝克型、埃默里-德赖富斯型、肢带型、面肩肱型肌营养不良症、肌肉梗死、多发性肌炎、运动神经元疾病和神经病变中均观察到未染色纤维。CoQ的组织化学检测方法特异性较低，因为在没有线粒体的区域如核心区域也存在部分染色。在所检查的19例线粒体肌病中均未观察到CoQ缺乏。
