现货，PTC124 (Ataluren)，selleck，美国进口，Others YZ剂 ，CAS号: 775304-57-9 ,目录号 S6003

PTC124 targets genetic disorders caused by nonsense mutations. ^[1] PTC124 is an orally bioavailable compound that promotes of the human CFTR-G542X nonsense allele in a CF mouse model. ^[2] Effectiveness of PTC124 treatment of cystic fibrosis caused by nonsense mutations: a prospective phase II trial. ^[3]
PTC124 induces dose-dependent suppression of premature translational termination without concomitant effects on normal termination or mRNA decay. ^[1]
In safety pharmacology studies in rats and dogs, oral administration of PTC124 induces no adverse neurological, pulmonary, or cardiovascular effects at doses through 1500 mg/kg. In toxicology studies in rats and dogs at oral doses through 1500 mg/kg for 28 days, PTC124 has shown good tolerability. ^[1]
The minimal concentration of PTC124 showing discernable readthrough was 0.01–0.1 mM (2.8–28 ng ml-1), whereas the concentration promoting maximal activity was approximately 3 mM(852 ng ml-1). ^[1]

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