CFTR nonsense mutations: Therapeutic benefits from clinically approved drugs?
Author:
Publisher
Elsevier BV
Subject
Pulmonary and Respiratory Medicine,Pediatrics, Perinatology and Child Health
Reference13 articles.
1. From CFTR biology toward combinatorial pharmacotherapy: expanded classification of cystic fibrosis mutations;Veit;Mol Biol Cell,2016
2. Atomic structure of the cystic fibrosis transmembrane conductance regulator;Zhang;Cell,2016
3. Cystic fibros is transmembrane conductance regulator (CFTR) potentiator VX-770 (ivacaftor) opens the defective channel gate of mutant CFTR in a phosphorylation-dependent but ATP-independent manner;Eckford;J Biol Chem,2012
4. Vx-770 potentiates CFTR function by promoting decoupling between the gating cycle and ATP hydrolysis cycle;Jih;Proc Natl Acad Sci U S A,2013
5. Cystic fibrosis genetics: from molecular understanding to clinical application;Cutting;Nat Rev Genet,2015
Cited by 2 articles. 订阅此论文施引文献 订阅此论文施引文献，注册后可以免费订阅5篇论文的施引文献，订阅后可以查看论文全部施引文献
1. CFTR mRNAs with nonsense codons are degraded by the SMG6-mediated endonucleolytic decay pathway;Nature Communications;2022-04-29
2. Nonsense-mediated RNA Decay Pathway Inhibition Restores Expression and Function of W1282X CFTR;American Journal of Respiratory Cell and Molecular Biology;2019-09
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