Role of Mutant CFTR in Hypersusceptibility of Cystic Fibrosis Patients to Lung Infections

作者：Gerald B. Pier, Martha Grout, Tanweer Zaidi, John C. Olsen, Larry G. Johnson, James R. Yankaskas, Joanna B. Goldberg · 发表于：Science · 年份：1996 · DOI：10.1126/science.271.5245.64 · 被引用次数：438 · 研究领域：Cystic Fibrosis Research Advances、Neonatal Respiratory Health Research、Bacterial biofilms and quorum sensing

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.