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Mitochondrial respiration impairment in MOCS1 deficient fibroblasts. OCR (A) was measured as detailed in the legend for Fig. 2. Basal respiration (B) and maximal respiration (C) of MOCS1 deficient fibroblasts treated or not with 40 nM JP4-039 (JP4) for 24 h. Data are means ± SD; number of replicates: 7–8. ***P < 0.001, ****P < 0.0001, compared to control (wild type) cells; #P < 0.05, ###P < 0.001 compared to MOCS1 deficient cells (Tukey multiple range test).
