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Figure 2 | Orphanet Journal of Rare Diseases

Figure 2

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

Figure 2

Primary skin fibroblasts of a normal control and of a patients with SCO2 mutations were incubated with 0, 100, 200, 400, and 600 μM BZF for 5 days and then assayed for COX activity. * = significant difference versus untreated (p<0.05). ** = significant versus 400 μM. Data are expressed normalized to protein, but there was no variation when they were normalized to CS activity.

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Orphanet Journal of Rare Diseases

ISSN: 1750-1172