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Figure 6 | Orphanet Journal of Rare Diseases

Figure 6

From: Copper and bezafibrate cooperate to rescue cytochrome c oxidase deficiency in cells of patients with sco2 mutations

Figure 6

Measurements of mitochondrial ATP concentration in control fibroblasts (A) and SCO2 cells (B). Where indicated the cells were challenged with 100 μM histamine, black and grey traces refer to BZF treated and untreated cells. The panel shows representative traces of 20 coverslips for each condition in 3 different experiments. Trace 1 (Tr1) = untreated control; Tr2 = control + 400 μM BZF; Tr3 = untreated SCO2 patient; Tr4 = SCO2 patient + 400 μM BZF.

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Orphanet Journal of Rare Diseases

ISSN: 1750-1172