VX-809 and Related Corrector Compounds Exhibit Secondary Activity Stabilizing Active F508del-CFTR after Its Partial Rescue to the Cell Surface-Reference-Cited by-同舟云学术

VX-809 and Related Corrector Compounds Exhibit Secondary Activity Stabilizing Active F508del-CFTR after Its Partial Rescue to the Cell Surface

Published:2014-05 Issue:5 Volume:21 Page:666-678
ISSN:1074-5521
Container-title:Chemistry & Biology
language:en
Short-container-title:Chemistry & Biology

Author:

Eckford Paul D.W.,Ramjeesingh Mohabir,Molinski Steven,Pasyk Stan,Dekkers Johanna F.,Li Canhui,Ahmadi Saumel,Ip Wan,Chung Timothy E.,Du Kai,Yeger Herman,Beekman Jeffrey,Gonska Tanja,Bear Christine E.

Publisher

Elsevier BV

Subject

Clinical Biochemistry,Drug Discovery,Pharmacology,Molecular Biology,Molecular Medicine,General Medicine,Biochemistry

Reference49 articles.

1. Effect of VX-770 in persons with cystic fibrosis and the G551D-CFTR mutation;Accurso;N. Engl. J. Med.,2010

2. Defective intracellular transport and processing of CFTR is the molecular basis of most cystic fibrosis;Cheng;Cell,1990

3. Results of a phase IIa study of VX-809, an investigational CFTR corrector compound, in subjects with cystic fibrosis homozygous for the F508del-CFTR mutation;Clancy;Thorax,2012

4. Cigarette smoke exposure induces CFTR internalization and insolubility, leading to airway surface liquid dehydration;Clunes;FASEB J.,2012

5. Electrophysiological, biochemical, and bioinformatic methods for studying CFTR channel gating and its regulation;Csanády;Methods Mol. Biol.,2011

Cited by 89 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. Comparison of a novel potentiator of CFTR channel activity to ivacaftor in ameliorating mucostasis caused by cigarette smoke in primary human bronchial airway epithelial cells;Respiratory Research;2024-07-10

2. Functional Consequences of CFTR Interactions in Cystic Fibrosis;International Journal of Molecular Sciences;2024-03-16

3. Recommended Tool Compounds for Modifying the Cystic Fibrosis Transmembrane Conductance Regulator Channel Variants;ACS Pharmacology & Translational Science;2024-03-14

4. Comparison of a novel potentiator of CFTR channel activity to ivacaftor in ameliorating mucostasis caused by cigarette smoke in primary human bronchial airway epithelial cells;2024-03-04

5. Organic Synthesis and Current Understanding of the Mechanisms of CFTR Modulator Drugs Ivacaftor, Tezacaftor, and Elexacaftor;Molecules;2024-02-10