Systemic deficiency of glutathione in cystic fibrosis. Academic Article uri icon

Overview

MeSH

  • Adult
  • Bronchoalveolar Lavage Fluid
  • Epithelial Cells
  • Epithelium
  • Extracellular Space
  • Female
  • Humans
  • Male
  • Oxidation-Reduction
  • Respiratory Function Tests

MeSH Major

  • Cystic Fibrosis
  • Glutathione

abstract

  • Cystic fibrosis (CF), a disorder characterized by mutations of the CF transmembrane regulator gene, is characterized in the lung by chronic inflammation, leading to progressive damage to the airway epithelium, bronchiectasis, and chronic obstructive lung disease. One process contributing to the airway derangement is the chronic burden of oxidants released by inflammatory cells on the respiratory epithelial surface. With this background, we hypothesized that glutathione in respiratory epithelial lining fluid (ELF) in CF patients might be oxidized and/or diminished in amount compared with that in normal subjects. Recovery of ELF by bronchoalveolar lavage from young adults with CF (n = 21) and normal subjects (n = 25) demonstrated marked neutrophil-dominated inflammation in ELF in CF patients. As predicted, ELF in CF patients was characterized by a deficiency of glutathione (P < 0.001), but this was secondary to a reduction in reduced glutathione (P < 0.001), inasmuch as there were no differences in ELF levels of oxidized glutathione (P > 0.2). Unexpectedly, there was also a marked deficiency of reduced glutathione in plasma (P < 0.02); i.e., the glutathione "deficiency" observed in ELF in CF patients is not limited to the site of the inflammation but is systemic. Although the etiology of this generalized deficiency of extracellular glutathione is unknown, it is important in considering options for treating the concomitant and devastating lung pathology in this disorder.

publication date

  • December 1993

has subject area

  • Adult
  • Bronchoalveolar Lavage Fluid
  • Cystic Fibrosis
  • Epithelial Cells
  • Epithelium
  • Extracellular Space
  • Female
  • Glutathione
  • Humans
  • Male
  • Oxidation-Reduction
  • Respiratory Function Tests

Research

keywords

  • Journal Article

Identity

Language

  • eng

PubMed ID

  • 8125859

Additional Document Info

start page

  • 2419

end page

  • 2424

volume

  • 75

number

  • 6