Yamamoto M, Nonaka I
Division of Ultrastructural Research, National Institute of Neuroscience, Tokyo, Japan.
Acta Neuropathol. 1988;76(6):558-63. doi: 10.1007/BF00689593.
Histochemical investigations were carried out on skeletal muscle biopsies from ten patients with chronic progressive external ophthalmoplegia with ragged-red fibers (RRF). In addition to the RRF, mild myopathic change consisting of variation in size of both type 1 and 2 fibers was seen in all patients, as well as neuropathic change in eight. Scattered fibers with absent cytochrome c oxidase (CCO) activity (focal deficiency) were seen in all patients. In serial sections, CCO deficiency did not always occupy the entire length of a fiber but was localized segmentally to regions measuring several hundred micrometers in length, suggesting the heterogeneity of CCO activity even in the same fiber.
对10例患有慢性进行性外眼肌麻痹伴破碎红纤维（RRF）的患者的骨骼肌活检组织进行了组织化学研究。除了RRF外，所有患者均可见1型和2型纤维大小变化组成的轻度肌病性改变，8例患者还存在神经病变。所有患者均可见散在的细胞色素c氧化酶（CCO）活性缺失的纤维（局灶性缺乏）。在连续切片中，CCO缺乏并不总是占据纤维的全长，而是节段性地定位于长度为几百微米的区域，这表明即使在同一纤维中CCO活性也存在异质性。
