Alterations in Calcium Handling Are a Common Feature in an Arrhythmogenic Cardiomyopathy Cell Model Triggered by Desmosome Genes Loss

Figures

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  Figure 1. Desmosomal expression levels of mRNA (A) and protein of the edited clones PKP2-KO (B), DSG2-KO (C), and DSC2-KO (D). * Indicates the gene expression levels that were significantly different between WT and edited clones; p-value < 0.05, see Table S4. Number of experiments: RT-PCR = 1; WB = 3.

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  Figure 2. Calcium cycle gene expression at the mRNA (A) and protein (B) levels. * Indicates the gene expression levels that were significantly different between WT and edited clones; p-value <0.05, see Table S4. Number of experiments: RT-PCR = 1; WB = 3.

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  Figure 3. Calcium imaging peaks (A) and studied parameters (B) of the three KO groups: PKP2-KO, DSG2-KO, and DSC2-KO. The black arrow in section A indicates the pulse of caffeine. * Indicates parameters that were significantly different compared with WT clones; p-value < 0.05, see Table S6.

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  Figure 4. Cx43 and SCN5A expression at the mRNA (A) and protein (B) levels of the three KO groups PKP2-KO, DSG2-KO, and DSC2-KO. * Indicates parameters that were significantly different compared with WT clones; p-value < 0.05, see Table S4. Number of experiments: RT-PCR = 1; WB = 3.

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  Figure 5. mRNA expression levels of several genes involved in ACM molecular pathways in the edited clones. * Indicates gene expression levels that were significantly different compared with WT clones; p-value < 0.05, see Table S8. Number of experiments: RT-PCR = 1.

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  Figure 6. mRNA expression levels of CACNA1C and PLN (A) and protein levels of DSC2, ANK2, Nav1.5, and DSG2 (B) in DSP clones. Number of experiments: RT-PCR = 1; WB = 1.

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  Figure 7. Calcium imaging peaks (A) and studied parameters (B) of the DSP clones. The black arrow in section A indicates the pulse of caffeine.