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. 2017 May 23;26(16):3056–3068. doi: 10.1093/hmg/ddx190

Figure 5.

Figure 5

ISOX and vorinostat partially restore splicing pattern in DM1 patient-derived fibroblasts. (A) ISOX and vorinostat partially rescue mis-splicing of SERCA and INSR in patient-derived DM1 fibroblasts. Human normal fibroblasts (N) and DM1 fibroblasts (DM1) were treated with DMSO (0.1%), ISOX (5 µM) and vorinostat (5 µM) for 2 days. SERCA exon 22 and INSR exon 11 splicing were analyzed by RT-PCR followed by gel electrophoresis. The percentage of exon inclusion is shown below each RT-PCR product. Biological triplicates/replicates of each treatment are indicated by 1, 2 and 3. (B, C) ImageJ quantification of the percent SERCA exon 22 inclusion (B) and INSR exon 11 inclusion (C) from three biological repeats of DM1 fibroblasts and two biological replicates of normal fibroblasts. Error bars show the standard deviation of the biological triplicates/replicates and statistical differences were characterized by a t test. *P ≤ 0.05; **P ≤ 0.01; ***P ≤ 0.001.