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Figure 2 (A) Frozen sections of skeletal muscle showing ragged red fibers indicated by arrows (a: Gomori's trichrome) that are strongly stained in the cytochrome oxidase (b) and succinic dehydrogenase (c) preparations. Fiber typing shows that these fibers are darkly stained type 1 fibers (d: ATPase pH 4.3). Bar in panel a represents 60 µm in panels a, b, c, and d. (B) Ultrastructural examination shows an excess of mitochondria and glycogen (arrow) at the periphery of a muscle fiber (a). Glycogen is sometimes seen within mitochondria (b, arrow). Bar in panel a represents 2 µm in panel a and 1 µm in panel b. (C) Histology from the postmortem myocardium of the 27-year-old patient with sudden cardiac death reveals coarse interstitial fibrosis, with extensive vacuolation of myocytes (arrow), marked pleomorphism of nuclei, and minimal disarray (H+E, x 200). Electron microscopy showed non-specific degenerative changes in mitochondria (not shown).





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