A nonaminoglycoside that has been reported to selectively induce ribosomes to read through premature nonsense stop signals on mRNA, thus allowing the production of full length, functional proteins (IC50 = 7 nM); 60 mg/kg s.c. injection or 0.3-0.9 mg/ml orally has been shown to restore CFTR protein expression and function in a mouse model of cystic fibrosis caused by nonsense mutations