Figure 7.

Dose-dependent inhibition of GAA•TTC•TTC repeat expansions in human HEK-293T cells by BQQ. (A) Baseline represents the frequency of the (GAA)₁₀₀•(TTC)₁₀₀ repeat expansion when bacterial pJC_GAA100 plasmid was transformed directly into yeast. Other columns show the frequency of GAA•TTC repeat expansions that occurred during plasmid replication in HEK-293T cells at two BQQ (benzoquinoquinoxaline) concentrations (20 and 40 nM). Error bars indicate standard error of the mean. Significance relative to the HEK-293T frequency value was determined using a two-way Welch ANOVA test (for 20 nM BQQ-OP concentration P value is ≤ 0.05 for 40 nM, P ≤ 0.005). (B) Chemical structure of Benzoquinoquinoxaline derivative (BQQ).