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. 2014 Sep 4;95(3):315–325. doi: 10.1016/j.ajhg.2014.08.003

Figure 2.

Figure 2

Morphological Findings

(A–C) The histochemical reaction to COX is diffusely decreased in muscle biopsies of individual S1 (A) and individual S2 (B), compared to a control biopsy (C). Scale bars represent 100 μm.

(D) Electron microscopy of muscle from individual S1 shows abnormal mitochondria with osmiophilic inclusions and cristae disarray. Scale bar represents 0.3 μm.

(E and F) Profound decrease of COX histochemical reaction is also visualized in fibroblasts from individual S6 (E) compared to a control cell line (F). Scale bars represent 10 μm.