Figure 1. Generation of DM1-iPSCs and their differentiation.

(A) The strategy of our study: patient iPSCs were passed and differentiated at three different passage numbers into CMs or neurons giving 9 samples (left), or had a MyoD1 vector transfected and were differentiated into myocytes, giving 6 samples (right). The CTG repeat lengths were measured in each sample. (B) Six clones from three different DM1 patients expressed pluripotent stem cell markers (Oct3/4, Nanog and Sox2) in conventional PCR. β-actin was used as a loading control. (C) Karyotypic analysis of undifferentiated iPSCS (Pt-1B). (D, left) Representative live image of CMs on day 20 (Pt-1B). A video clip is available in Supplementary Video 1. (D, right) FACS analysis of the CMs shown in the picture on the left. The X-axis indicates the percentage of cardiac troponin T (cTnT)-positive cells among the total number of CMs. The Y-axis indicates the autofluorescence of the CMs. (E) Representative immunostaining image of neurons on day 42 (Pt-1B). The left panel shows neurons that expressed Tyrosine Hydroxylase (TH) and Microtubule-associated protein 2 (Map2). The right panel shows neurons that expressed TH and Neuron-specific Class III β-tubulin (TUJ1). (F) Representative immunostaining image of myocytes on day 7 (Pt-1B). The myocytes expressed Myosin Heavy Chain (MHC). Hoechst stains the nuclei.