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An experimental model of mitochondrial myopathy: germanium-induced myopathy and coenzyme Q10 administration
C M Wu1, T Matsuoka, M Takemitsu
1Division of Ultrastructural Research, National Center of Neurology and Psychiatry, Tokyo, Japan.
Abstract:
In skeletal muscles from rats treated with germanium for 23 weeks, there were numerous ragged-red fibers and cytochrome-c oxidase (COX)-deficient fibers. Biochemically, germanium reduced the enzyme activities in the mitochondrial respiratory chain. Rotenone-sensitive NADH-cytochrome-c reductase as well as COX activities were markedly reduced, while succinate-cytochrome-c reductase was less severely, but significantly, affected. The histopathological findings in these muscles were similar to those seen in patients with mitochondrial encephalomyopathy, suggesting that germanium-induced myopathy may be a useful experimental model. Coenzyme Q10 administration appeared to be ineffective in preventing this experimental myopathy.