The bidirectional relationship between CFTR and lipids

Commun Biol. 2020 Apr 20;3(1):179. doi: 10.1038/s42003-020-0909-1.

Abstract

Cystic Fibrosis (CF) is the most common life-shortening genetic disease among Caucasians, resulting from mutations in the gene encoding the Cystic Fibrosis Transmembrane conductance Regulator (CFTR). While work to understand this protein has resulted in new treatment strategies, it is important to emphasize that CFTR exists within a complex lipid bilayer - a concept largely overlooked when performing structural and functional studies. In this review we discuss cellular lipid imbalances in CF, mechanisms by which lipids affect membrane protein activity, and the specific impact of detergents and lipids on CFTR function.

Publication types

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

MeSH terms

  • Aminophenols / therapeutic use
  • Aminopyridines / therapeutic use
  • Animals
  • Benzodioxoles / therapeutic use
  • Cystic Fibrosis / drug therapy
  • Cystic Fibrosis / genetics
  • Cystic Fibrosis / metabolism*
  • Cystic Fibrosis / physiopathology
  • Cystic Fibrosis Transmembrane Conductance Regulator / drug effects
  • Cystic Fibrosis Transmembrane Conductance Regulator / genetics
  • Cystic Fibrosis Transmembrane Conductance Regulator / metabolism*
  • Drug Combinations
  • Epithelial Cells / drug effects
  • Epithelial Cells / metabolism*
  • Humans
  • Lung / drug effects
  • Lung / metabolism*
  • Lung / physiopathology
  • Membrane Lipids / metabolism*
  • Membrane Microdomains / metabolism*
  • Mutation
  • Protein Conformation
  • Protein Stability
  • Protein Transport
  • Quinolones / therapeutic use
  • Structure-Activity Relationship

Substances

  • Aminophenols
  • Aminopyridines
  • Benzodioxoles
  • CFTR protein, human
  • Drug Combinations
  • Membrane Lipids
  • Quinolones
  • lumacaftor, ivacaftor drug combination
  • Cystic Fibrosis Transmembrane Conductance Regulator
  • ivacaftor
  • lumacaftor