Role of mutant CFTR in hypersusceptibility of cystic fibrosis patients to lung infections

Science. 1996 Jan 5;271(5245):64-7. doi: 10.1126/science.271.5245.64.


Cystic fibrosis (CF) patients are hypersusceptible to chronic Pseudomonas aeruginosa lung infections. Cultured human airway epithelial cells expressing the delta F508 allele of the cystic fibrosis transmembrane conductance regulator (CFTR) were defective in uptake of P. aeruginosa compared with cells expressing the wild-type allele. Pseudomonas aeruginosa lipopolysaccharide (LPS)-core oligosaccharide was identified as the bacterial ligand for epithelial cell ingestion; exogenous oligosaccharide inhibited bacterial ingestion in a neonatal mouse model, resulting in increased amounts of bacteria in the lungs. CFTR may contribute to a host-defense mechanism that is important for clearance of P. aeruginosa from the respiratory tract.

Publication types

  • Research Support, U.S. Gov't, P.H.S.

MeSH terms

  • Animals
  • Animals, Newborn
  • Cell Line, Transformed
  • Cystic Fibrosis / complications*
  • Cystic Fibrosis / genetics
  • Cystic Fibrosis / microbiology
  • Cystic Fibrosis Transmembrane Conductance Regulator / genetics
  • Cystic Fibrosis Transmembrane Conductance Regulator / physiology*
  • Disease Susceptibility
  • Epithelium / microbiology
  • Humans
  • Lipopolysaccharides / pharmacology
  • Lung / microbiology
  • Mice
  • Mice, Inbred BALB C
  • Pseudomonas Infections / etiology*
  • Pseudomonas Infections / microbiology
  • Pseudomonas aeruginosa / physiology*
  • Respiratory System / microbiology*
  • Respiratory Tract Infections / etiology*
  • Respiratory Tract Infections / microbiology


  • CFTR protein, human
  • Lipopolysaccharides
  • Cystic Fibrosis Transmembrane Conductance Regulator