IDH2 R140Q inhibitor AGI-6780 reverses DNA hypermethylation. (A-C) R140Q expression results in a DNA hypermethylation phenotype (A, combined clones 11 and 17; B, clone 11; C, clone 17) that can be reversed by 1 μM AGI-6780 treatment of 7 or 8 days (D, left; E) or 28 days (D, middle), with a concomitant decrease in 2HG from 21.44 mM (TF-1 R140Q) to 0.44 mM (TF-1 R140Q + AGI-6780). Venn diagram (inset) shows overlap between methylation sites increased with IDH2 R140Q expression and decreased on AGI-6780 treatment (red circle). A smaller number of DMPs are induced by AGI-6780 treatment of pLVX cells (D, right). Significance, q-value <.05; minimum β-value difference, 0.1.