Pyrimido[4,5-b]quinoline-4,5(3H,10H)-dione, 2-cyclobutyl-10-methyl-3-phenyl-

SRI-41315 is a small molecule that induces translational readthrough of CFTR nonsense mutations by eRF1 depletion. SRI-41315 induce a prolonged pause at stop codons and suppress PTCs associated with cystic fibrosis in immortalized and primary human bronchial epithelial cells, restoring CFTR expression and function. SRI-41315 suppresses PTCs by reducing the abundance of the termination factor eRF1. SRI-41315 also potentiates aminoglycoside-mediated readthrough, leading to synergistic increases in CFTR activity.