ISSN:
1432-0533
Keywords:
Mitochondrial encephalomyopathy
;
Myoclonus epilepsy with ragged-red fibers (MERRF)
;
Strongly succinate dehydrogenase-reactive blood vessels (SSV)
;
Cytochrome c oxidase (CCO)
;
Vascular involvement
Source:
Springer Online Journal Archives 1860-2000
Topics:
Medicine
Notes:
Summary More than half of the intramuscular blood vessels in muscle biopsies from five patients with myoclonus epilepsy with ragged-fibers (MERRF) who had a point mutation in mitochondrial DNA at the tRNALys region were darkly stained with succinate dehydrogenase (SDH) stain, showing the morphologic characteristics of strongly SDH-reactive blood vessels (SSV), but they had no cytochrome c oxidase (CCO) activity. By electron cytochemistry, the mitochondria in the smooth muscle cells of SSV had no CCO activity. On the other hand, SSV in muscle biopsies from patients with mitochondrial myopathy, encephalopathy, lactic acidosis, and stroke-like episodes (MELAS) had normal CCO activity as shown by light and electron microscopy. The defect in CCO activity in the arteriolar smooth muscle cells and in muscle fibers suggests that CCO deficiency is related to the pathophysiology of MERRF.
Type of Medium:
Electronic Resource
URL:
http://dx.doi.org/10.1007/BF00227723
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