Two Small Molecules Restore Stability to a Subpopulation of the Cystic Fibrosis Transmembrane Conductance Regulator with the Predominant Disease-causing Mutation-Reference-Cited by-同舟云学术

Two Small Molecules Restore Stability to a Subpopulation of the Cystic Fibrosis Transmembrane Conductance Regulator with the Predominant Disease-causing Mutation

Published:2017-03 Issue:9 Volume:292 Page:3706-3719
ISSN:0021-9258
Container-title:Journal of Biological Chemistry
language:en
Short-container-title:Journal of Biological Chemistry

Author:

Meng Xin,Wang Yiting,Wang Xiaomeng,Wrennall Joe A.,Rimington Tracy L.,Li Hongyu,Cai Zhiwei,Ford Robert C.,Sheppard David N.

Funder

Cystic Fibrosis Foundation Therapeutics

Cystic Fibrosis Trust

Medical Research Council

Engineering and Physical Sciences Research Council

Publisher

Elsevier BV

Subject

Cell Biology,Molecular Biology,Biochemistry

Reference77 articles.

1. Cystic fibrosis;Ratjen;Nat. Rev. Dis. Primers,2015

2. Origins of cystic fibrosis lung disease;Stoltz;N. Engl. J. Med,2015

3. Identification of the cystic fibrosis gene: cloning and characterization of complementary DNA;Riordan;Science,1989

4. The ABC protein turned chloride channel whose failure causes cystic fibrosis;Gadsby;Nature,2006

5. Physiology of epithelial chloride and fluid secretion;Frizzell;Cold Spring Harb. Perspect. Med,2012

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