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PTC124 (Ataluren)

Cat No.
CEI-0252
Description
PTC124 is a inhibitor of Firefly luciferase (FLuc) with IC50 of 7 nM.
CAS No.
775304-57-9
Molecular Weight
284.24
Purity
>99%
Storage
2 years at -20centigrade Powder
Targets
Firefly luciferase (FLuc)
Molecular Formula
C15H9FN2O3
Chemical Name
3-(5-(2-fluorophenyl)-1,2,4-oxadiazol-3-yl)benzoic acid
Solubility
DMSO 57 mg/mL Water
In vitro
PTC124 is a inhibitor of Firefly luciferase (FLuc) with IC50 of 7 nM, but PTC124 can also increase cellular FLuc activity levels by posttranslational stabilization. FLuc inhibition and stabilization is the result of an inhibitory product formed during the FLuc-catalyzed reaction between its natural substrate, ATP, and PTC124. And the inhibitory activity of PTC124-AMP is relieved by free coenzyme A. PTC124 can effectively suppress the hCFTR-G542X nonsense mutation in vivo. Oral administration of PTC124 to Cftr-/- mice expressing a human CFTR-G542X transgene suppressed the G542X nonsense mutation and restored a significant amount of human (h)CFTR protein and function.
In vivo
PTC124 treatment resulted in the appearance of hCFTR protein at the apical surface of intestinal glands in Cftr-/- hCFTR-G542X mice, and the treatment also restored 24-29% of the average cAMP-stimulated transepithelial chloride currents observed in wild-type mice.
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