Salt-independent abnormality of antimicrobial activity in cystic fibrosis airway surface fluid

Am J Respir Cell Mol Biol. 2001 Jul;25(1):21-5. doi: 10.1165/ajrcmb.25.1.4436.


The link between the genetic defect in cystic fibrosis (CF) and the recently described breach in pulmonary host defense has focused on the role of salt and water metabolism in the airways. Using a human bronchial xenograft model we demonstrate a salt-independent abnormality in bacterial killing in CF airway surface fluid (ASF). Biochemical characterization implicates the absence or dysfunction of a molecule critical to the constitution of normal bacterial killing. Our study suggests that CF transmembrane conductance regulator (CFTR) deficiency causes a primary abnormality in the composition of ASF that leads to a salt-independent defect in host defense. Importantly, this defect is corrected by adenovirus-mediated gene transfer of CFTR.

Publication types

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

MeSH terms

  • Adolescent
  • Adult
  • Antibodies / immunology
  • Bronchoalveolar Lavage Fluid / chemistry*
  • Bronchoalveolar Lavage Fluid / microbiology
  • Child
  • Child, Preschool
  • Cystic Fibrosis / metabolism*
  • Cystic Fibrosis / microbiology
  • Cystic Fibrosis Transmembrane Conductance Regulator / genetics*
  • Cystic Fibrosis Transmembrane Conductance Regulator / metabolism
  • Humans
  • Infant
  • Sodium Chloride / metabolism*
  • Transplantation, Heterologous
  • beta-Defensins / immunology
  • beta-Defensins / physiology*


  • Antibodies
  • CFTR protein, human
  • beta-Defensins
  • Cystic Fibrosis Transmembrane Conductance Regulator
  • Sodium Chloride