SRI-37240 is a potent premature termination codons (PTCs) inhibitor. SRI-37240 suppresses CFTR nonsense mutations. SRI-37240 alters cellular translation termination at PTCs in HEK293T cells. SRI-37240 can also restore CFTR function in primary bronchial epithelial cells when combination with G418[1].
SRI-37240 (1, 3, 10 and 30 µM; 48 h) induces concentration-dependent increases in CFTR-dependent (Forskolin-stimulated and sensitive to the inhibitor CFTRInh-172) chloride conductance[1].
SRI-37240 (10 µM; 72 h) significantly increases the amount of full-length, fully glycosylated form of CFTR protein, and the unprocessed, immature form of full-length CFTR protein in 16HBEge cells when co-treated with G418 (100 µM)[1].
SRI-37240 (10 µM; 24 h) alters cellular translation termination at PTCs in HEK293T cells, also increases global densities of ribosomes at normal stop codons without affecting densities of ribosomes in 3-UTRs[1].
SRI-37240 (10 µM; 72 h) restores CFTR function in primary bronchial epithelial cells when combination with G418[1].
Western Blot Analysis[1]
| Cell Line: | CFTR-G542X 16HBEge |
| Concentration: | 10 µM |
| Incubation Time: | 24 h |
| Result: | Significantly increased the amount of Band C CFTR protein, which represents the full-length, fully glycosylated form of CFTR and Band B, which represents the unprocessed, immature form of full-length CFTR protein when combined with G418 (100 µM). |
[1]. Sharma J, et al. A small molecule that induces translational readthrough of CFTR nonsense mutations by eRF1 depletion. Nat Commun. 2021 Jul 16;12(1):4358.