Fig. 2.

CQ treatment of DM1 iPDMs improves phenotypes. (A–E) Fluorescence images of LC3 immunostaining (red) of iCDMs (control [CNT]) (A) and iPDMs (B) differentiated for 96 h and treated with vehicle or CQ (C–E) for 48 (Scale bar, 20 μm.) (F–G) Analysis of colocalization efficiency (F) and Manders’ coefficient (G) between MBNL1 and (CAG)₇ in iPDMs treated with CQ 0.1 and 10 µM. Colocalization efficiencies were normalized to untreated iPDMs (H₂O) and data were expressed as percentage (%) in the case of colocalization efficiency. Between 50 and 60 nuclei were analyzed. (H) RT-qPCR analysis to quantify expression of DMPK relative to GAPDH in iPDMs after CQ treatment (n = 3). (I–J) Quantification of myotube diameter and myogenic fusion index of iCDMs (green) and untreated iPDMs (red) and iPDMs treated with CQ (blue) (n = 7 to 10 images per condition). (K–O) Representative confocal images of Desmin-immunostained (green) human myoblasts transdifferentiated for 7 d used for quantifications in I and J. Staining was performed in iCDMs (K), and iPDMs untreated (L) or treated with CQ (M–O) for 48 h. (Scale bar, 1,000 µm.) Nuclei were counterstained with DAPI (blue). **P < 0.01, ***P < 0.001 according to Student’s t test. All comparisons refer to untreated iPDMs (H₂O in the graphs).