Research Paper Volume 12, Issue 7 pp 6260—6275

Myotonic Dystrophy type 1 cells display impaired metabolism and mitochondrial dysfunction that are reversed by metformin

Figure 6. Metformin restores OXPHOS activity and ROS production in DM1 fibroblasts. (A) Representative kinetic normalized OCR response in DM1 (n=6) and control fibroblasts (n=3) after treatment with 1 μM of metformin for 72 h. DM1 and control fibroblasts were plated at 5.000 cells/well 24 hours prior to the assay. A representative experiment out of 3 is shown. (BD) Quantification of mitochondrial basal respiration, maximal respiration, and ATP production respectively after treatment with 1 μM of metformin for 72 h of controls (n=3) and DM1 fibroblasts (n=6). (E) mRNA levels of OPA1, MFN1, MFN2, DRP1 and TFAM after treatment with 1 μM of metformin for 72 h (n≥2). (F) H2O2 production after treatment with 1 μM of metformin for 72 h (n=3). (G) mRNA levels of SIRT1, GPX1 and PARKIN in DM1 and control fibroblasts after treatment with 1 μM of metformin 72 h (n≥2).