Fig. 6
FUS silencing via ION363 treatment ex vivo in IPF PCLs downregulates the expression of multiple profibrotic RNAs. a Representative immunoblot images of FUS and GAPDH in IPF PCLs treated with either scramble ASO (Scr) or the indicated doses of ION363 (ASO) for 72 h. Quantification revealed a significant decrease in FUS protein upon ION363 treatment at 15 and 20 µg. b PCA plot showing a distinct pattern between Scr and ASO/ION363 treatments. c A Z-score heatmap plot of the top 50 DEGs is shown. d Gene set enrichment analysis using kobas for the Reactome database, showing differential regulation of genes associated with the extracellular matrix with the highest significance. All the ECM-related groups are highlighted in red. e Gene set enrichment via the KEGG disease database showing genes related to skin and soft tissue diseases and genes related to respiratory diseases (red font). f Volcano plot of genes whose expression was upregulated (red) or downregulated (blue) in IPF PCLs upon ION363 treatment. Genes that are associated with the greatest log2FC or FDR (XIST1, LRP2; black font) and genes associated with the alveolar/respiratory epithelium (red font) are also depicted. All analyses were performed on PCLs derived from n = 3 IPF patients/group
