The cystic fibrosis airway milieu enhances rescue of F508del in a pre-clinical model

Eur Respir J. 2018 Dec 20;52(6):1801133. doi: 10.1183/13993003.01133-2018. Print 2018 Dec.
No abstract available

Publication types

  • Letter
  • Research Support, N.I.H., Extramural
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Aminophenols / pharmacology
  • Aminopyridines / pharmacology
  • Benzodioxoles / pharmacology
  • Bronchi / cytology*
  • Bronchi / drug effects
  • Cell Line
  • Colforsin / pharmacology
  • Cystic Fibrosis / genetics*
  • Cystic Fibrosis / physiopathology*
  • Cystic Fibrosis Transmembrane Conductance Regulator / genetics*
  • Epithelial Cells / cytology*
  • Epithelial Cells / drug effects
  • Humans
  • Inflammation
  • Mutation
  • Quinolones / pharmacology
  • Respiratory System

Substances

  • Aminophenols
  • Aminopyridines
  • Benzodioxoles
  • Quinolones
  • cystic fibrosis transmembrane conductance regulator delta F508
  • Cystic Fibrosis Transmembrane Conductance Regulator
  • Colforsin
  • ivacaftor
  • lumacaftor