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Figure 2 Histochemical analysis of skeletal muscle and cardiac tissue. (A) Age-matched control heart sample dual-stained for cytochrome c oxidase (COX) and succinate dehydrogense (SDH) activity. (B) Right ventricular sample from Patient no. IV-03 (family 1) dual-stained for COX and SDH activity, highlighting the distribution of COX-negative (blue) cells. (C) Left ventricular sample from Patient no. IV-03 (family 1) dual-stained for COX and SDH activity. (D) Left ventricular sample from Patient no. IV-01 (family 2) dual-stained for COX and SDH activity. (E) A transverse section of skeletal muscle from the proband in family 1 (IV-03) reacted for COX activity, showing a normal staining pattern. The scale bar represents 200 µm.
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