Figure 5.

Investigation of allele-specificity in cells with equal levels of mutant and wild-type COCH transcription

Mutant COCH-minigene-expressing cells from a clone with comparable expression levels of endogenous wild-type COCH were transfected with AONs. (A) Transfection of cells with c.151C>T AON-E results in a dose-depended decrease in mutant COCH transcript levels. Allele-specific reduction of mutant COCH transcript levels was observed at 25 nM but not at 100 and 250 nM. (B) Mutant and wild-type COCH transcript levels in cells treated with 25 nM c.151C>T AON-E, dupAG AON-B, or scrambled control AON. Mutant and wild-type COCH transcript levels are normalized for the expression of RPS18 plotted as mean ± SD or 3 replicates. ∗∗∗p < 0.001, two-way ANOVA with Tukey’s multiple-comparison post-test conducted for possible comparisons. Outcome for relevant comparisons is shown; asterisks indicate the p value compared to tetracycline-treated cells.