Potentiator ivacaftor abrogates pharmacological correction of ΔF508 CFTR in cystic fibrosis-Reference-Cited by-同舟云学术

Potentiator ivacaftor abrogates pharmacological correction of ΔF508 CFTR in cystic fibrosis

Published:2014-07-23 Issue:246 Volume:6 Page:
ISSN:1946-6234
Container-title:Science Translational Medicine
language:en
Short-container-title:Sci. Transl. Med.

Author:

Cholon Deborah M.¹,Quinney Nancy L.¹,Fulcher M. Leslie¹,Esther Charles R.¹²,Das Jhuma³,Dokholyan Nikolay V.³,Randell Scott H.¹⁴⁵,Boucher Richard C.¹⁴,Gentzsch Martina¹⁵

Affiliation:

1. Marsico Lung Institute/Cystic Fibrosis Research Center, University of North Carolina at Chapel Hill, Chapel Hill, NC 27599, USA.

2. Division of Pediatric Pulmonology, Department of Pediatrics, University of North Carolina at Chapel Hill, Chapel Hill, NC 27599, USA.

3. Department of Biochemistry and Biophysics, University of North Carolina at Chapel Hill, Chapel Hill, NC 27599, USA.

4. Department of Medicine, University of North Carolina at Chapel Hill, Chapel Hill, NC 27599, USA.

5. Department of Cell Biology and Physiology, University of North Carolina at Chapel Hill, Chapel Hill, NC 27599, USA.

Abstract

Ivacaftor, a CFTR potentiator drug used for cystic fibrosis, destabilizes rescued ΔF508 CFTR and interferes with the action of drugs that correct CFTR function.

Publisher

American Association for the Advancement of Science (AAAS)

Subject

General Medicine

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