Fig. 1 | Scientific Reports

Fig. 1

From: Selective activation of antioxidant resources and energy deficiency in Marinesco–Sjögren syndrome fibroblasts as an adaptive biological response to Sil1 loss

Fig. 1

Energetic profile of MSS fibroblasts measured with Seahorse using ATP rate assay. (A–C) Control fibroblasts (CT) and patient-derived fibroblasts (MSS) energetic map and ATP production from mitochondrial and glycolytic pathways. The histograms show comparable levels of ATP from the glycolytic pathway and a decrease in ATP from oxidative phosphorylation in MSS cells. (D) Quantification of total ATP production shows a decrease in MSS cells. (E,F) OCR and PER graphs show both decreased levels in MSS cell. (G) OCR measurements in cells treated with FCCP showed reduced maximal mitochondrial respiration in patient-derived fibroblasts. Statistical analysis was conducted using an unpaired t-test with Welch’s correction (***P < 0.001).

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