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CFTR transmembrane segments are impaired in their conformational adaptability by a pathogenic loop mutation and dynamically stabilized by Lumacaftor

The cystic fibrosis transmembrane conductance regulator (CFTR) is an ion channel protein that is defective in individuals with cystic fibrosis (CF). To advance the rational design of CF therapies, it is important to elucidate how mutational defects in CFTR lead to its impairment and how pharmacologi...

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Detalhes bibliográficos
Publicado no:J Biol Chem
Main Authors: Krainer, Georg, Schenkel, Mathias, Hartmann, Andreas, Ravamehr-Lake, Dorna, Deber, Charles M., Schlierf, Michael
Formato: Artigo
Idioma:Inglês
Publicado em: American Society for Biochemistry and Molecular Biology 2020
Assuntos:
Acesso em linha:https://ncbi.nlm.nih.gov/pmc/articles/PMC7029128/
https://ncbi.nlm.nih.gov/pubmed/31882543
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1074/jbc.AC119.011360
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