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. Author manuscript; available in PMC: 2021 Feb 20.
Published in final edited form as: Cell Chem Biol. 2020 Jan 24;27(2):223–231.e4. doi: 10.1016/j.chembiol.2020.01.003

Figure 4: Biological activity of 2 in DM2 patient-derived fibroxblasts.

Figure 4:

(A) Representative microscopic images for the reduction of nuclear foci by 2, as completed by RNA-FISH and immunohistochemistry using an anti-MBNL1 antibody. (B) Quantification of r(CCUG)exp-MBNL1 foci/nucleus (n = 3 biological replicates, 40 nuclei counted per replicate). (C) Effect of 2 on IR pre-mRNA splicing regulated by MBNL1. (D) Effect of 2 on CNBP pre-mRNA splicing. (E) Effect of 2 on an MBNL1- regulated alternative splicing event, IR exon 11, as determined by RT-PCR; (F) Effect of 2 on aberrant CNBP pre-mRNA splicing caused by r(CCUG)exp, as determined by RT-PCR; that is, retention of intron 1; (G) Effect of 2 on total intron 1 levels and CNBP mature mRNA, as determined by RT-qPCR. Error bars represent SD. *P < 0.5, **P < 0.01, ***P < 0.001, as determined by a one-way ANOVA (n = 3). See also Figure S3.