Figure 1 .

RAN translation is upregulated by dsRNA and ER stress. Stable cell lines expressing nanoluciferase-tagged QAGR (A) or LPAC (B) proteins from constructs containing CAGG or CCUG repeats, respectively, were transfected with double-stranded polyI:C RNA (5 μg/ml) or treated with thapsigargin (Tg) (1 μM) for 12 hrs. Upper graphs show relative luminescence units (RLU) of cells with and without polyIC or Tg treatment. Middle panels show protein blots of treated and untreated cells probed with antibodies against phospho-eIF2α (p-eIF2α), total eIF2α and GAPDH. Lower panels show quantification of p-eIF2α/eIF2α. Error bars show standard deviation (SD). n = 3. Statistical analyses were performed using the two-tailed t-test, ^*P < 0.05, ^**P < 0.01, ^****P < 0.0001.