SRI-37240 is a potent inhibitor of premature termination codons (PTCs) with read-across activity that induces a prolonged pause at the termination codon and inhibits PTCs associated with cystic fibrosis.SRI-37240 can be used to study PTC-related diseases.

SRI-37240 (1, 3, 10, and 30 μM; 48 h) enhances CFTR-dependent chloride conductance in a concentration-dependent manner, activated by Forskolin and inhibited by CFTR Inh-172 [1]. At 10 μM over 72 h, it significantly increases levels of both mature, fully glycosylated and immature CFTR protein forms in 16HBEge cells, especially when used with G418 (100 μM) [1]. Additionally, at 10 μM for 24 h, SRI-37240 alters cellular translation termination at premature termination codons (PTCs) in HEK293T cells, boosts ribosome densities at normal stop codons, and does not affect ribosome densities in 3'-UTRs [1]. The compound also restores CFTR function in primary bronchial epithelial cells with G418 treatment over 72 h [1]. Western Blot Analysis shows a significant increase in both mature (Band C) and immature (Band B) CFTR protein forms at 10 μM for 24 h in CFTR-G542X 16HBEge cells with G418 (100 μM) [1].