ISSN:
1432-0533
Keywords:
Mitochondrial encephalomyopathies
;
Cytochrome c oxidase
;
Muscle culture
;
Electron cytochemistry
;
Tissue specificity
Source:
Springer Online Journal Archives 1860-2000
Topics:
Medicine
Notes:
Summary The populations of cytochrome c oxidase (CCO)-positive and-negative mitochondria were analyzed in the elongated cells containing occasional multiple nuclei (myotubes) in primary muscle cultures derived from patients with various forms of mitochondrial encephalomyopathies with CCO deficiency. Even in control muscle cultures, CCO-positive (79.7%) and -negative (20.3%) mitochondria were distributed randomly, showing intracellular mosaicism. All mitochondria in all muscle cultures from two patients with clinical characteristics of Leigh's disease exhibited faint to negative CCO activity. In these patients no enzyme activity could be detected in any tissue including intrafusal fibers and fibroblasts in muscle biopsies. In patients with the fatal infantile and the encephalomyopathic forms of CCO deficiency, and myoclonic epilepsy with ragged-red fibers, two different types of myotubes containing mostly CCO-positive mitochondria and only negative mitochondria, respectively, representing intercellular mosaicism, were demonstrated. The intercellular mosaicism in biopsied and cultured muscles in the case of CCO deficiency supports the contention that both CCO-positive and -negative mitochondria coexist in the early myogenic cell and later randomly segregated during cell division (mitotic segregation), forming two different cells.
Type of Medium:
Electronic Resource
URL:
http://dx.doi.org/10.1007/BF00308814
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