Figure 1.

Ataluren significantly increases microRNA (miR) processing across a range of nonsense pulmonary arterial hypertension (PAH) mutations. Cells were treated with 3 μM ataluren for 24 hours, then the appropriate bone morphogenetic protein (BMP) ligand for 4 hours. RNA was analyzed for miR-27a, normalized to RNU48. Results are shown as fold change of miR-27a with BMP treatment compared with vehicle. (A) Pulmonary artery endothelial cells (PAECs; R321X and R294X) or late-outgrowth endothelial progenitor cells (L-EPCs; W9X) treated with BMP9. (B) Lymphoblastoid cell line (LCL) treated with BMP4. Means (±SEM) of nine replicates are shown. *P < 0.05; **P < 0.001.