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 2. Correlation between impaired metabolism and markers of disease pathophysiology. (A) Quantification of protein levels shown in Figure 1F (n=4). (B) mRNA levels of DMPK in DM1 fibroblasts (n=7) and controls (n=3). (C) Basal respiration levels in controls (n=3) and DM1 fibroblasts stratified by CTG expansion in <500 (n=4) and >500 (n=3) (left) and MIRS scale in <3 (n=2) and >3 n=5 (right). (D) ATP production levels using the same stratification.