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. 2011 Dec 20;135(1):62–71. doi: 10.1093/brain/awr326

Figure 3.

Figure 3

High levels of COX-deficiency, presence of mitochondrial DNA deletions and evidence of reduced mitochondrial DNA copy number in Patient 3 dorsal root ganglia neurons. (A) Patient COX/succinate dehydrogenase histochemistry reveals 60% neuronal COX-deficiency (COX/succinate dehydrogenase histochemistry, blue cells). (B) Molecular analysis of patient dorsal root ganglia tissues shows the mean level of mitochondrial DNA deletions is slightly higher in COX-deficient neurons versus COX-positive neurons. (C) Succinate dehydrogenase histochemistry on patient dorsal root ganglia neurons reveals some variability in activity between neurons (succinate dehydrogenase histochemistry), with some neurons demonstrating a high level of succinate dehydrogenase activity (D) and some with only a low level of succinate dehydrogenase activity (E). (F) MT-ND1 copy number: evidence of mitochondrial DNA depletion is shown by a significant reduction in absolute copy number with MT-ND1 in patient neurons relative to controls (Mann–Whitney test, P = 0.003). (G) MT-ND4 copy number: wild-type mitochondrial DNA copy number, as assessed by MT-ND4, is significantly lower in patient neurons relative to control neurons (Mann–Whitney test, P < 0.0001), indicative of presence of mitochondrial DNA deletions in the patient's dorsal root ganglia neurons.