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Iron accumulates in the lavage and explanted lungs of cystic fibrosis patients.
Ghio, Andy, V. Roggli, J. Soukup, J. Richards, S. Randell, AND M. Muhlebach. Iron accumulates in the lavage and explanted lungs of cystic fibrosis patients. Journal of Cystic Fibrosis. Elsevier BV, AMSTERDAM, Netherlands, 12:S1569-1993, (2012).
Oxidative stress, associated with a disruption in iron homeostasis, may participate in the pathophysiology of cystic fibrosis disease. We tested this hypothesis by looking at both bronchoalveolar lavage and explanted lung tissues from patients with cystic fibrosis.
Abstract 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 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.