| Online-Ressource |
Verfasst von: | Neves, Joana [VerfasserIn]  |
| Leitz, Dominik [VerfasserIn]  |
| Agrawal, Raman [VerfasserIn]  |
| Mall, Marcus A. [VerfasserIn]  |
| Altamura, Sandro [VerfasserIn]  |
| Muckenthaler, Martina [VerfasserIn]  |
Titel: | Disruption of the Hepcidin/Ferroportin regulatory system causes pulmonary iron overload and restrictive lung disease |
Verf.angabe: | Joana Neves, Dominik Leitz, Simone Kraut, Christina Brandenberger, Raman Agrawal, Norbert Weissmann, Christian Mühlfeld, Marcus A. Mall, Sandro Altamura, Martina U. Muckenthaler |
E-Jahr: | 2017 |
Jahr: | 29 April 2017 |
Umfang: | 10 S. |
Fussnoten: | Gesehen am 04.09.2017 |
Titel Quelle: | Enthalten in: EBioMedicine |
Ort Quelle: | Amsterdam [u.a.] : Elsevier, 2014 |
Jahr Quelle: | 2017 |
Band/Heft Quelle: | 20(2017), Seite 230-239 |
ISSN Quelle: | 2352-3964 |
Abstract: | 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. |
DOI: | doi:10.1016/j.ebiom.2017.04.036 |
URL: | Bitte beachten Sie: Dies ist ein Bibliographieeintrag. Ein Volltextzugriff für Mitglieder der Universität besteht hier nur, falls für die entsprechende Zeitschrift/den entsprechenden Sammelband ein Abonnement besteht oder es sich um einen OpenAccess-Titel handelt.
Kostenfrei: Volltext ; Verlag: http://dx.doi.org/10.1016/j.ebiom.2017.04.036 |
| Kostenfrei: Volltext: http://www.sciencedirect.com/science/article/pii/S2352396417301913 |
| DOI: https://doi.org/10.1016/j.ebiom.2017.04.036 |
Datenträger: | Online-Ressource |
Sprache: | eng |
Sach-SW: | Ferroportin |
| Hepcidin resistance |
| Hereditary hemochromatosis |
| Iron Overload |
| Restrictive lung disease |
K10plus-PPN: | 1563016567 |
Verknüpfungen: | → Zeitschrift |
Disruption of the Hepcidin/Ferroportin regulatory system causes pulmonary iron overload and restrictive lung disease / Neves, Joana [VerfasserIn]; 29 April 2017 (Online-Ressource)