Ataluren-13C6

Ataluren-13C6 (PTC124-13C6) is 13C labeled Ataluren. Ataluren (PTC124) is an orally available CFTR-G542X nonsense allele inhibitor.

Product Specifications

Product Name Alternative

PTC124-13C6

UNSPSC

12352005

Target

Autophagy; CFTR; Isotope-Labeled Compounds

Related Pathways

Autophagy; Membrane Transporter/Ion Channel; Others

Applications

Metabolism-protein/nucleotide metabolism

Field of Research

Metabolic Disease; Cancer

Smiles

O=C(C1=CC=CC(C2=NOC([13C]3=[13C][13C]=[13C][13C]=[13C]3F)=N2)=C1)O

Molecular Formula

C9 13C6H5FN2O3

Molecular Weight

286.17

References & Citations

[1]Russak EM, et al. Impact of Deuterium Substitution on the Pharmacokinetics of Pharmaceuticals. Ann Pharmacother. 2019;53 (2) :211-216.|[2]Welch EM, et al. PTC124 targets genetic disorders caused by nonsense mutations. Nature, 2007, 447 (7140), 87-91.|[3]Pettit RS, et al. CFTR Modulators for the Treatment of Cystic Fibrosis. P T. 2014 Jul;39 (7) :500-11.|[4]Miller JN, et al. The novel Cln1 (R151X) mouse model of infantile neuronal ceroid lipofuscinosis (INCL) for testing nonsense suppression therapy. Hum Mol Genet. 2015 Jan 1;24 (1) :185-96.