Extended Data Fig. 9: enDUB-U21 restores functional currents of N1303K and F508del CFTR mutant channels in combination with Trikafta.
From: Targeted deubiquitination rescues distinct trafficking-deficient ion channelopathies
a, Population I-V curves for forskolin-activated currents from FRT cells stably expressing WT CFTR (black circles, n = 57) and N1303K + CFP (red squares, n = 8), and forskolin-activated, VX770-potentiated currents from N1303K + CFP treated with VX661 + VX445 (green triangles, n = 9); N1303K + enDUB-U21CF.E3h treated with VX661 + VX445 (blue triangles, n = 9); n cells examined over ≥3 independent experiments (mean ± s.e.m). Data for N1303K + CFP adapted from Fig. 5. b, Population I-V curves for forskolin-activated currents from FRT cells stably expressing WT CFTR (black circles, n = 57) and F508del + CFP (red squares, n = 12), and forskolin-activated, VX770-potentiated currents from F508del + CFP treated with VX661 + VX445 (green triangles, n = 10); F508del + enDUB-U21CF.T2a treated with VX661 + VX445 (blue triangles, n = 9); n cells examined over ≥3 independent experiments (mean ± s.e.m). Data for F508del + CFP adapted from Fig. 6. *p < 0.03, **p < 0.009,***p < 0.0001, two-way ANOVA with Tukey’s multiple comparison test.
