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 1. DM1-derived fibroblasts present impaired metabolism. (A) Kinetic normalized OCR response in DM1 and control fibroblasts in basal conditions and after consecutive addition of Oligomycin 1.5 μM, FCCP 1.5 μM and Antimycin-A/Rotenone 1.5 μM. A representative experiment out of 3 is shown with 3 independent control cultures and 2 DM1. (B, C) Quantification of mitochondrial respiratory functions and coupling efficiency in DM1 (n=7) and control fibroblasts (n=3). (D) Representative energy map and (E) Quantification of metabolic potential of DM1 and control fibroblasts. Stressed indicates the values of OCR and ECAR after the injection of oligomycin and FCCP simultaneously. Results are obtained from controls (n=3) and DM1 (n=5) cultures. (F) Representative immunoblots of phospho-AKT, AKT, DMPK and MBNL1 in DM1-derived fibroblasts and healthy controls (n=3).