Mosaicism of mitochondria in mitochondrial myopathy : an electronmicroscopic analysis of cytochrome c oxidase

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...

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Bibliographic Details
Published in:Acta neuropathologica 1990-10, Vol.80 (6), p.642-648
Main Authors: HAGINOYA, K, MIYABAYASHI, S, IINUMA, K, TADA, K
Format: Article
Language:English
Subjects:
Adult
Biological and medical sciences
Child
Diseases of striated muscles. Neuromuscular diseases
Electron Transport Complex IV - immunology
Electron Transport Complex IV - ultrastructure
Female
Histocytochemistry
Humans
Immunohistochemistry
Infant, Newborn
Medical sciences
Microscopy, Electron
Mitochondria, Muscle - enzymology
Mitochondria, Muscle - ultrastructure
Muscles - enzymology
Muscles - pathology
Muscles - ultrastructure
Muscular Diseases - enzymology
Muscular Diseases - pathology
Neurology
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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.
ISSN:0001-6322
1432-0533
DOI:10.1007/BF00307633