• Iron In Lung Pathology

    From ironjustice@21:1/5 to All on Mon May 14 07:43:53 2018
    Disruption of the Hepcidin/Ferroportin Regulatory System Causes Pulmonary Iron Overload and Restrictive Lung Disease.
    Neves J1, Leitz D2, Kraut S3, Brandenberger C4, Agrawal R2, Weissmann N3, Mühlfeld C4, Mall MA5, Altamura S6, Muckenthaler MU7.
    EBioMedicine. 2017 Jun;20:230-239. doi: 10.1016/j.ebiom.2017.04.036. Epub 2017 Apr 29.

    Emerging evidence suggests that pulmonary iron accumulation is implicated in a spectrum of chronic lung diseases. However, the mechanism(s) involved in pulmonary iron deposition and its role in the in vivo pathogenesis of lung diseases remains unknown.
    Here we show that a point mutation in the murine ferroportin gene, which causes hereditary hemochromatosis type 4 (Slc40a1C326S), increases iron levels in alveolar macrophages, epithelial cells lining the conducting airways and lung parenchyma, and in
    vascular smooth muscle cells. Pulmonary iron overload is associated with oxidative stress, restrictive lung disease with decreased total lung capacity and reduced blood oxygen saturation in homozygous Slc40a1C326S/C326S mice compared to wild-type
    controls. These findings implicate iron in lung pathology, which is so far not considered a classical iron-related disorder.

    Ferroportin; Hepcidin resistance; Hereditary hemochromatosis; Iron Overload; Restrictive lung disease

    Comment in
    Does Pathological Iron Overload Impair the Function of Human Lungs? [EBioMedicine. 2017]
    PMID: 28499927 PMCID: PMC5478206 DOI: 10.1016/j.ebiom.2017.04.036

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