Mosaicism of mitochondria in mitochondrial myopathy: an electronmicroscopic analysis of cytochrome c oxidase |
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Authors: | K. Haginoya S. Miyabayashi K. Iinuma K. Tada |
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Affiliation: | (1) Department of Pediatrics, Tohoku University School of Medicine, 1-1 Seiryo-machi, Aoba-ku, 980 Sendai, Japan |
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Abstract: | Summary Electron microscopic histochemistry was applied to the study of cytochrome c oxidase activity in each mitochondrion of biopsied muscles from four patients with mitochondrial myopathy [one case of fatal infantile mitochondrial myopathy, one case of myoclonus epilepsy associated with ragged-red fibers (MERRF), and two cases of mitochondrial myopathy, encephalopathy, lactic acidosis and stroke-like episodes (MELAS)]. In the patient with fatal infantile mitochondrial myopathy, intercellular heterogeneity of mitochondria was recognized. In the three patients with either MERRF or MELAS, cytochrome c oxidase activity was segmentally changed from positive to negative within single muscle fibers. In the two patients with MELAS, small groups of positive-stained mitochondria were located among negative-stained mitochondria in the negative segment of a few muscle fibers. These findings revealed that there were heterogeneous populations of normal and abnormal mitochondria intracellularly or intercellularly within the muscles of these patients.Supported in part by Grant-in-Aid for Scientific Research 63570422 from the Ministry of Education, Science and Culture, and Grant 62A-5-08 from the National Center of Neurology and Psychiatry (NCNP) of the Ministry of Health and Welfare, Japan |
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Keywords: | Mitochondrial myopathy Cytochrome c oxidase Mosaicism of mitochondria Electron microscopic histochemistry |
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