235 Fatty Acid Oxidation in PLN R14del Cardiomyopathy 8 Supplementary Figure 7 can be downloaded from: https://assets.researchsquare.com/files/ rs-1902254/v1/35bc8505201af85fabf9b6d4.png Supplementary Figure 7: A) Representative immunofluorescence images showing the well developed mitochondrial organization (stained by Tomm20 in red) and sarcomere (stained by α-actinin in green) in long-termed cultured control and PLN-R14del hiPSC-Cms. Nuclei were stained by DAPI (blue). B) Seahorse XF Cell Mito Stress Test demonstrated a lower oxygen consumption rate (OCR) in PLN-R14del hiPSC-CMs than the controls, suggesting an impaired mitochondrial function in PLN-R14del hiPSC-CMs. Supplementary Figure 8 can be downloaded from: https://assets.researchsquare.com/files/ rs-1902254/v1/d16d47c689fca3a5d93dd40b.png Supplementary Figure 8: A) Enrichment analysis using downregulated genes in PLN-R14del versus control hiPSC-CMs cultured in the maturation medium pointed towards altered metabolic-related biological functions. B) Enrichment analysis using downregulated genes in PLNR14del versus control hiPSC-CMs cultured in the glucose-rich medium pointed towards altered metabolic-related biological functions. C) Enrichment analysis using downregulated genes in PLN-R14del versus control hiPSC-CMs cultured in the lipid-rich medium pointed towards altered metabolic-related biological functions. D) STRING protein-protein-interaction network highlighting oxidative phosphorylation-related protein-coding genes (red) that were downregulated in PLNR14del hiPSC-CMs cultured in the maturation medium. E) STRING protein-protein-interaction network highlighting oxidative phosphorylation-related protein-coding genes (red) that were downregulated in PLN-R14del hiPSC-CMs cultured in the glucose-rich medium. F) STRING protein-protein-interaction network highlighting oxidative phosphorylation-related protein-coding genes (red) that were downregulated in PLN-R14del hiPSC-CMs cultured in the lipid-rich medium.
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