Mutation-Specific Phenotypes in hiPSC-Derived Cardiomyocytes Carrying Either Myosin-Binding Protein C Or α-Tropomyosin Mutation for Hypertrophic Cardiomyopathy

Figure 3

Arrhythmogenic events (DADs and EADs) were observed in HCM-CMs. (a)–(e) Representative recordings of control hiPSC-derived CMs (WT) and hiPSC-derived CMs carrying TPM1-Asp175Asn (HCMT) or MYBPC3-Gln1061X (HCMM) mutations. Typical DADs (arrows) are presented in (b) and (c) and EADs (arrows) in (d) and (e) for HCMT-CMs and HCMM-CMs, respectively. Scale bars represent 40 mv and 5 seconds, respectively. Scale bars in (a) are representative for (b) and (c), and scale bars in (d) are representative for (e). (f) Distribution of CMs exhibiting arrhythmogenic events in each cell line. (g) DAD rate was significantly higher in HCMM-CMs than in WT-CMs ().

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