Novel Adult-Onset Systolic Cardiomyopathy Due to MYH7 E848G Mutation in Patient-Derived Induced Pluripotent Stem Cells
Kai-Chun Yang, Astrid Breitbart, Willem J. De Lange, Peter Hofsteen, Akiko Futakuchi-Tsuchida, Joy Xu, Cody Schopf, Maria V. Razumova, Alex Jiao, Robert Boucek, Lil Pabon, Hans Reinecke, Deok-Ho Kim, J. Carter Ralphe, Michael Regnier and Charles E. Murry
Characterization of Contractile Function and Calcium Transients in hiPSC-CMs
(A) No difference in maximum averaged normalized shortening in day 30 WT-CMs and FCM-CMs. (B, C) Impaired maximum fractional shortening is evident in day 50 FCM-CMs compared with age-matched WT-CMs. (N = 3 WT lines and N = 3 FCM lines, n > 10 cells for each day 30 line; Kruskal-Wallis test, p > 0.05) (N = 3 WT lines and N = 3 FCM lines, n > 21 for each day 50 line; Kruskal-Wallis test, p < 0.05; *p < 0.05 with 2-tailed Mann-Whitney U test). (D) Normalized averaged calcium transients in day 50 human induced pluripotent stem cell–derived cardiomyocyte (hiPSC-CMs) are not significantly different. All cells paced at 1.5 Hz. Error bars indicate SEM. See Supplemental Videos 1 and 2. Abbreviations as in Figures 1 and 2.