Iron accumulates in the lavage and explanted lungs of cystic fibrosis patients.

Journal Article (Journal Article)

Oxidative stress participates in the pathophysiology of cystic fibrosis (CF). An underlying disruption in iron homeostasis can frequently be demonstrated in injuries and diseases associated with an oxidative stress. We tested the hypothesis that iron accumulation and altered expression of iron-related proteins could be demonstrated in both the bronchoalveolar lavage (BAL) fluid and explanted lungs of patients with cystic fibrosis. BAL fluid collected from 10 children with CF showed elevated concentrations of protein, iron, ferritin, transferrin, heme, and hemoglobin relative to that obtained from 20 healthy volunteers. Using Perl's Prussian blue staining, explanted lung from CF patients revealed increased iron in alveolar and interstitial macrophages. Similarly, there was an increased expression of ferritin, the iron importer DMT1, and the exporter ferroportin 1 in lung tissue from CF patients. We conclude that iron homeostasis is disrupted in CF patients with an accumulation of this metal and altered expression of iron-related proteins being evident in the lungs.

Full Text

Duke Authors

Cited Authors

  • Ghio, AJ; Roggli, VL; Soukup, JM; Richards, JH; Randell, SH; Muhlebach, MS

Published Date

  • July 2013

Published In

Volume / Issue

  • 12 / 4

Start / End Page

  • 390 - 398

PubMed ID

  • 23176785

Electronic International Standard Serial Number (EISSN)

  • 1873-5010

Digital Object Identifier (DOI)

  • 10.1016/j.jcf.2012.10.010


  • eng

Conference Location

  • Netherlands