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. Author manuscript; available in PMC: 2022 Jun 27.
Published in final edited form as: J Am Coll Cardiol. 2020 Mar 17;75(10):1159–1174. doi: 10.1016/j.jacc.2019.12.066

FIGURE 1. The DGC in hiPSC-Derived CMs and Human Cardiac Samples in the Presence and Absence of Dystrophin.

FIGURE 1

(A) Schematic of the DGC. (B, C) qRT-PCR time course of Dp427m (full-length muscle dystrophin gene) and MYL2 genes in control hiPSC-derived CMs (n = 3). (D) Western blot expression of the DGC components in hiPSC-derived CMs and adult human LV. These results showed that the day 60 hiPSC-derived CMs expressed the protein components of the DGC and sarcomeric proteins (n = 3). (E) WGA pulldown of control and DMD hiPSC-derived CM lines at day 60 differentiation demonstrated that the DGC components were all expressed and pulled down in hiPSC-derived CMs and adult human LV. In the DMD hiPSC-derived CMs at day 60, the absence of dystrophin leads to the lack of nucleation of the DGC. Cypher was also absent in DMD hiPSC-derived CMs (n = 3). (F) Control human LV samples showed the expression and pulldown of dystrophin, whereas an absence of dystrophin was seen in the 2 human DMD LV samples (n = 3). The DMD human LV samples have a lack of nucleation of the DGC, including the absence of cypher. CM = cardiomyocyte; Con = control; D = day; DGC = dystrophin-glycoprotein complex; DMD = Duchenne muscular dystrophy; GAPDH = glyceraldehyde 3-phsophate dehydrogenase; hiPSC = human induced pluripotent-derived cardiomyocytes; LV = left ventricle; qRT-PCR = quantitative real-time polymerase chain reaction; WGA = wheat germ agglutinin.