Potentiator ivacaftor abrogates pharmacological correction of ΔF508 CFTR in cystic fibrosis
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- Deborah M. Cholon
- Marsico Lung Institute/Cystic Fibrosis Research Center, University of North Carolina at Chapel Hill, Chapel Hill, NC 27599, USA.
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- Nancy L. Quinney
- Marsico Lung Institute/Cystic Fibrosis Research Center, University of North Carolina at Chapel Hill, Chapel Hill, NC 27599, USA.
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- M. Leslie Fulcher
- Marsico Lung Institute/Cystic Fibrosis Research Center, University of North Carolina at Chapel Hill, Chapel Hill, NC 27599, USA.
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- Charles R. Esther
- Marsico Lung Institute/Cystic Fibrosis Research Center, University of North Carolina at Chapel Hill, Chapel Hill, NC 27599, USA.
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- Jhuma Das
- Department of Biochemistry and Biophysics, University of North Carolina at Chapel Hill, Chapel Hill, NC 27599, USA.
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- Nikolay V. Dokholyan
- Department of Biochemistry and Biophysics, University of North Carolina at Chapel Hill, Chapel Hill, NC 27599, USA.
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- Scott H. Randell
- Marsico Lung Institute/Cystic Fibrosis Research Center, University of North Carolina at Chapel Hill, Chapel Hill, NC 27599, USA.
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- Richard C. Boucher
- Marsico Lung Institute/Cystic Fibrosis Research Center, University of North Carolina at Chapel Hill, Chapel Hill, NC 27599, USA.
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- Martina Gentzsch
- Marsico Lung Institute/Cystic Fibrosis Research Center, University of North Carolina at Chapel Hill, Chapel Hill, NC 27599, USA.
説明
<jats:p>Ivacaftor, a CFTR potentiator drug used for cystic fibrosis, destabilizes rescued ΔF508 CFTR and interferes with the action of drugs that correct CFTR function.</jats:p>
収録刊行物
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- Science Translational Medicine
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Science Translational Medicine 6 (246), 246ra96-, 2014-07-23
American Association for the Advancement of Science (AAAS)