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 3. DM1-derived fibroblasts have no changes in mitochondria biogenesis. (A, B) Representative images of immunofluorescence of TOMM20, and PGC1-α in DM1 and control fibroblasts (n=3). (C) Medium fluorescence intensity of MitoTracker Red FM in control (n=3) and DM1 cells (n=5) and (D) of Rhodamine 123 in DM1 and control fibroblasts (n=3). (E) mRNA levels of TFAM transcription factor (n=3). (F) mRNA levels of OPA1, MFN1, MFN2, DRP1 and PARKIN in DM1 and control fibroblasts (n≥2).