Cystic fibrosis (CF), Pseudomonas aeruginosa, CFTR and the CF glycosylation phenotype: A review and update

L. I. Stoykova, T. F. Scanlin

Research output: Contribution to journalReview article

1 Scopus citations

Abstract

Cystic Fibrosis (CF) is a common, ultimately fatal, inherited disorder of exocrine glands. The dominant clinical feature is a chronic progressive lung disease mediated by airways infection and inflammation. The most important pathogen in the lungs is Pseudomonas aeruginosa (P. aeruginosa). Eventually more than 80 percent of all CF individuals are chronically infected with this organism. The responsible gene, named the CF Transmembrane Regulator (CFTR), was identified in 1989. Although our understanding of the disease has increased, treatment is symptomatic and the details of pathogenesis at the molecular level remain to be elucidated. The decreased conductance of chloride at the surface of airway epithelial cells is the most characteristic pathophysiologic abnormality and is the target of much of the research to develop new therapies. Shortly after the identification of the sweat chloride defect in CF, biochemical analysis of mucins defined an altered carbohydrate composition of CF mucins when compared to controls. The altered terminal glycosylation of CF glycoproteins has been described by many investigators, although there is not a consensus. Since the recent determination of the crystal structure of the major binding protein of P. aeruginosa - (PA-IIL) and the demonstration of its high affinity binding to the same fucosylated oligosaccharides (Lewis x and Lewis a) that are characteristic of the CF glycophenotype, there has been a renewed interest in the role of altered terminal glycosylation in the pathogenesis of CF. This review will provide an update of studies on the altered terminal glycosylation in CF and will discuss the possible role of glycosylation in the pathogenesis of the pulmonary infection in CF and its link to CFTR function.

Original languageEnglish (US)
Pages (from-to)900-910
Number of pages11
JournalCurrent Organic Chemistry
Volume12
Issue number11
DOIs
StatePublished - Jul 1 2008
Externally publishedYes

All Science Journal Classification (ASJC) codes

  • Organic Chemistry

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