Abstract
Several clinical syndromes have been associated with cytochrome oxidase (EC 1.9.3.1) deficiency. Mitochondrial encephalomyopathy, lactic acidosis and stroke-like episodes (MELAS) might be one such syndrome (Pavlakis et al., 1984). Here we present a patient with a progressive encephalomyopathy, stroke-like episodes and cytochrome oxidase deficiency in the biopsied skeletal muscle.
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References
Hatefi, Y. and Rieske, J. S. The preparation and properties of DPNH-cytochrome c reductase (Complex I-III of the respiratory chain). Methods Enzymol. 10 (1967) 225–231
Hasuo, K., Tamura, S., Yasumori, K., Uchino, A., Goda, S., Ishimoto, S., Kamikaseda, K., Wakuta, Y., Kishi, M. and Masuda, K. Computed tomography and angiography in MELAS (mitochondrial myopathy, encephalopathy, lactic acidosis and stroke-like episodes); report of 3 cases, Neuroradiology 29 (1987) 393–397
King, T. E. Preparation of succinate dehydrogenase and reconstitution of succinate oxidase. Methods Enzymol 10 (1967) 322–331
King, T. E. and Howard, R. L. Preparations and properties of soluble NADH dehydrogenases from cardiac muscle. Methods Enzymol 10 (1967) 275–294
Pavlakis, S. G., Phillips, P. C., Di Mauro, S., De Vivo, D. C. and Rowland, L. P. Mitochondrial myopathy, encephalopathy, lactic acidosis and stroke-like episodes: a distinctive clinical syndrome. Ann. Neurol 16 (1984) 481–488
Sanadi, D. R., Pharo, R. L. and Sordahl, L. A. NADH-CoQ reductase — Assay and purification. Methods Enzymol 10 (1967) 297–302
Tisdale, H. D. Preparation and properties of succinic-cytochrome c reductase (Complex II-III). Methods Enzymol 10 (1967) 213–225
Wharton, D. C. and Tzagoloff, A. Cytochrome oxidase from beef heart mitochondria. Methods Enzymol 10 (1967) 245–250
Ziegler, D. and Rieske, J. S. Preparation and properties of succinate dehydrogenase- coenzyme Q reductase (Complex II). Methods Enzymol 10 (1967) 231–234
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© 1988 Springer Science+Business Media Dordrecht
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Maertens, P., Richardson, R., Bastian, F., Williams, J.P., Hommes, F. (1988). A New Type of Mitochondrial Encephalomyopathy with Stroke-like Episodes due to Cytochrome Oxidase Deficiency. In: Pollitt, R.J., Harkness, R.A., Addison, G.M. (eds) Studies in Inherited Metabolic Disease. Springer, Dordrecht. https://doi.org/10.1007/978-94-009-1259-5_27
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DOI: https://doi.org/10.1007/978-94-009-1259-5_27
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