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. Author manuscript; available in PMC: 2021 Feb 21.
Published in final edited form as: ACS Chem Biol. 2020 Jan 13;15(2):485–493. doi: 10.1021/acschembio.9b00958

Figure 4.

Figure 4.

Biological activity of 2 in DM2 fibroblasts. (A) Effect of 2 on r(CCUG)exp-containing CNBP levels measured by RT-qPCR. (B) Effect of 2 on CNBP mature mRNA levels measured by RT-qPCR. (C) Effect of competitive assay between 1 and 2 on CNBP mature mRNA levels measured by RT-qPCR. (D) Ability of 2 to rescue the IR exon 11 splicing defect. (E) Representative microscopic images for the reduction of nuclear foci by 2, as completed by RNA-FISH and immunohistochemistry (IHC) using an anti-MBNL1 antibody.32 (F) Quantification of r(CCUG)exp-MBNL1 foci/nucleus (n = 3 biological replicates, 40 nuclei counted per replicate). Error bars represent SD. *P < 0.5, **P < 0.01, ***P < 0.001, ****P < 0.0001 as determined by a one-way ANOVA (n = 3).