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Research ArticleCystic Fibrosis
Potentiator ivacaftor abrogates pharmacological correction of ΔF508 CFTR in cystic fibrosis
- Deborah M. Cholon1,
- Nancy L. Quinney1,
- M. Leslie Fulcher1,
- Charles R. Esther Jr.1,2,
- Jhuma Das3,
- Nikolay V. Dokholyan3,
- Scott H. Randell1,4,5,
- Richard C. Boucher1,4 and
- Martina Gentzsch1,5,*
- 1Marsico Lung Institute/Cystic Fibrosis Research Center, University of North Carolina at Chapel Hill, Chapel Hill, NC 27599, USA.
- 2Division of Pediatric Pulmonology, Department of Pediatrics, University of North Carolina at Chapel Hill, Chapel Hill, NC 27599, USA.
- 3Department of Biochemistry and Biophysics, University of North Carolina at Chapel Hill, Chapel Hill, NC 27599, USA.
- 4Department of Medicine, University of North Carolina at Chapel Hill, Chapel Hill, NC 27599, USA.
- 5Department of Cell Biology and Physiology, University of North Carolina at Chapel Hill, Chapel Hill, NC 27599, USA.
- ↵*Corresponding author. E-mail: gentzsch{at}med.unc.edu
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Science Translational Medicine 23 Jul 2014:
Vol. 6, Issue 246, pp. 246ra96
DOI: 10.1126/scitranslmed.3008680
Vol. 6, Issue 246, pp. 246ra96
DOI: 10.1126/scitranslmed.3008680
Deborah M. Cholon
1Marsico Lung Institute/Cystic Fibrosis Research Center, University of North Carolina at Chapel Hill, Chapel Hill, NC 27599, USA.
Nancy L. Quinney
1Marsico Lung Institute/Cystic Fibrosis Research Center, University of North Carolina at Chapel Hill, Chapel Hill, NC 27599, USA.
M. Leslie Fulcher
1Marsico Lung Institute/Cystic Fibrosis Research Center, University of North Carolina at Chapel Hill, Chapel Hill, NC 27599, USA.
Charles R. Esther Jr.
1Marsico Lung Institute/Cystic Fibrosis Research Center, University of North Carolina at Chapel Hill, Chapel Hill, NC 27599, USA.
2Division of Pediatric Pulmonology, Department of Pediatrics, University of North Carolina at Chapel Hill, Chapel Hill, NC 27599, USA.
Jhuma Das
3Department of Biochemistry and Biophysics, University of North Carolina at Chapel Hill, Chapel Hill, NC 27599, USA.
Nikolay V. Dokholyan
3Department of Biochemistry and Biophysics, University of North Carolina at Chapel Hill, Chapel Hill, NC 27599, USA.
Scott H. Randell
1Marsico Lung Institute/Cystic Fibrosis Research Center, University of North Carolina at Chapel Hill, Chapel Hill, NC 27599, USA.
4Department of Medicine, University of North Carolina at Chapel Hill, Chapel Hill, NC 27599, USA.
5Department of Cell Biology and Physiology, University of North Carolina at Chapel Hill, Chapel Hill, NC 27599, USA.
Richard C. Boucher
1Marsico Lung Institute/Cystic Fibrosis Research Center, University of North Carolina at Chapel Hill, Chapel Hill, NC 27599, USA.
4Department of Medicine, University of North Carolina at Chapel Hill, Chapel Hill, NC 27599, USA.
Martina Gentzsch
1Marsico Lung Institute/Cystic Fibrosis Research Center, University of North Carolina at Chapel Hill, Chapel Hill, NC 27599, USA.
5Department of Cell Biology and Physiology, University of North Carolina at Chapel Hill, Chapel Hill, NC 27599, USA.
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