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Folding–function relationship of the most common cystic fibrosis–causing CFTR conductance mutants

Marcel van Willigen, Annelotte M Vonk, Hui Ying Yeoh, Evelien Kruisselbrink, Bertrand Kleizen, Cornelis K van der Ent, Maarten R Egmond, Hugo R de Jonge, View ORCID ProfileIneke Braakman, Jeffrey M Beekman, View ORCID ProfilePeter van der Sluijs  Correspondence email
Marcel van Willigen
1Cellular Protein Chemistry, Department of Chemistry, Utrecht University, Utrecht, The Netherlands
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Annelotte M Vonk
2Department of Pediatric Pulmonology, Wilhelmina Children's Hospital, University Medical Center, Utrecht, The Netherlands
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Hui Ying Yeoh
1Cellular Protein Chemistry, Department of Chemistry, Utrecht University, Utrecht, The Netherlands
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Evelien Kruisselbrink
2Department of Pediatric Pulmonology, Wilhelmina Children's Hospital, University Medical Center, Utrecht, The Netherlands
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Bertrand Kleizen
1Cellular Protein Chemistry, Department of Chemistry, Utrecht University, Utrecht, The Netherlands
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Cornelis K van der Ent
2Department of Pediatric Pulmonology, Wilhelmina Children's Hospital, University Medical Center, Utrecht, The Netherlands
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Maarten R Egmond
1Cellular Protein Chemistry, Department of Chemistry, Utrecht University, Utrecht, The Netherlands
3Membrane Biochemistry and Biophysics, Department of Chemistry, Utrecht University, Utrecht, The Netherlands
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Hugo R de Jonge
4Department of Gastroenterology and Hepatology, Erasmus University Medical Center, Rotterdam, The Netherlands
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Ineke Braakman
1Cellular Protein Chemistry, Department of Chemistry, Utrecht University, Utrecht, The Netherlands
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  • ORCID record for Ineke Braakman
Jeffrey M Beekman
2Department of Pediatric Pulmonology, Wilhelmina Children's Hospital, University Medical Center, Utrecht, The Netherlands
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Peter van der Sluijs
1Cellular Protein Chemistry, Department of Chemistry, Utrecht University, Utrecht, The Netherlands
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  • ORCID record for Peter van der Sluijs
  • For correspondence: p.vandersluijs1@uu.nl
Published 18 January 2019. DOI: 10.26508/lsa.201800172
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Folding–function relationship of CF mutants
Marcel van Willigen, Annelotte M Vonk, Hui Ying Yeoh, Evelien Kruisselbrink, Bertrand Kleizen, Cornelis K van der Ent, Maarten R Egmond, Hugo R de Jonge, Ineke Braakman, Jeffrey M Beekman, Peter van der Sluijs
Life Science Alliance Jan 2019, 2 (1) e201800172; DOI: 10.26508/lsa.201800172

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Folding–function relationship of CF mutants
Marcel van Willigen, Annelotte M Vonk, Hui Ying Yeoh, Evelien Kruisselbrink, Bertrand Kleizen, Cornelis K van der Ent, Maarten R Egmond, Hugo R de Jonge, Ineke Braakman, Jeffrey M Beekman, Peter van der Sluijs
Life Science Alliance Jan 2019, 2 (1) e201800172; DOI: 10.26508/lsa.201800172
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Volume 2, No. 1
February 2019
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  • Cell Biology
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Cited By...

  • Rectal organoid-guided CFTR modulator therapy restores lung function in a cystic fibrosis patient with the rare 1677delTA/R334W genotype
  • ABC-transporter CFTR folds with high fidelity through a modular, stepwise pathway
  • Co-translational folding of the first transmembrane domain of ABC-transporter CFTR is supported by assembly with the first cytosolic domain
  • Fluorescence assay for simultaneous quantification of CFTR ion-channel function and plasma membrane proximity
  • Fluorescence assay for simultaneous quantification of CFTR ion-channel function and plasma membrane proximity
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