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. 2019 Sep 2;9:12651. doi: 10.1038/s41598-019-49014-2

Figure 7.

Figure 7

Elevated superoxide levels in ETHE1 and MOCS1 deficient fibroblasts are reduced by JP4-039. Superoxide levels were evaluated in ETHE1-1, ETHE1-2, ETHE1-3, ETHE1-4 and MOCS1 deficient fibroblasts treated with JP4-039 (JP4) (A–E) or in ETHE1-4 treated with N-acetylcysteine (NAC) (F). ETHE1 deficient cells were treated with JP4 (40 or 200 nM) or NAC (0.5 and 1 µM) during 24 h and incubated with MitoSOX Red. MOCS1 deficient fibroblasts were treated with JP4-039 (40 or 200 nM) for 7 days and incubated with MitoSOX Red. Data are presented as mean ± SD; number of replicates: 3–4. *P < 0.05, **P < 0.01, ***P < 0.001, ****P < 0.0001, compared to control (wild type) cells; #P < 0.05, ##P < 0.01, compared to patient cells (Tukey multiple range test).