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

Figure 5

Cardiac-specific protein expression in hiPSC-derived CMs. (a) Representative images of hiPSC-derived CMs carrying TPM1-Asp175Asn mutation (HCMT) or MYBPC3-Gln1061X mutation (HCMM) and hiPSC-derived control CMs (WT) stained with cTnT, MYBPC, and TPM1. These images are not to quantify the protein expression but to demonstrate the presence of cTnT, MYBPC, and TPM1 proteins in the hiPSC-derived CMs. (b) hiPSC-derived CMs from all cell lines expressed MYBPC, cTnT, and TPM1 proteins. The truncated MYBPC was not detected in HCMM cells (size of the wild type protein 141 kDa and the predicted size of the truncated protein 117 kDa). (c) The expression of MYBPC, cTnT, and TPM1 in WT-CMs, HCMT-CMs, and HCMM-CMs normalized to the expression of β-actin. Protein expressions were quantified from western blots using ImageJ software. Quantitation data show the averages of MYBPC/β-actin, cTnT/β-actin, and TPM1/β-actin relations from hiPSC-derived CMs from two different hiPSC lines in each group. Because of the lack of replicates, statistical analysis was not performed.

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