Disruption of the Hepcidin/Ferroportin regulatory system causes pulmonary iron overload and restrictive lung disease
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 murin...
Gespeichert in:
| Hauptverfasser: | , , , , , |
|---|---|
| Dokumenttyp: | Article (Journal) |
| Sprache: | Englisch |
| Veröffentlicht: |
29 April 2017
|
| In: |
EBioMedicine
Year: 2017, Jahrgang: 20, Pages: 230-239 |
| ISSN: | 2352-3964 |
| DOI: | 10.1016/j.ebiom.2017.04.036 |
| Online-Zugang: | Verlag, kostenfrei, Volltext: http://dx.doi.org/10.1016/j.ebiom.2017.04.036 Verlag, kostenfrei, Volltext: http://www.sciencedirect.com/science/article/pii/S2352396417301913 
|
| Verfasserangaben: | Joana Neves, Dominik Leitz, Simone Kraut, Christina Brandenberger, Raman Agrawal, Norbert Weissmann, Christian Mühlfeld, Marcus A. Mall, Sandro Altamura, Martina U. Muckenthaler |
MARC
| LEADER | 00000caa a2200000 c 4500 | ||
|---|---|---|---|
| 001 | 1563016567 | ||
| 003 | DE-627 | ||
| 005 | 20230427122046.0 | ||
| 007 | cr uuu---uuuuu | ||
| 008 | 170904s2017 xx |||||o 00| ||eng c | ||
| 024 | 7 | |a 10.1016/j.ebiom.2017.04.036 |2 doi | |
| 035 | |a (DE-627)1563016567 | ||
| 035 | |a (DE-576)493016562 | ||
| 035 | |a (DE-599)BSZ493016562 | ||
| 035 | |a (OCoLC)1340979090 | ||
| 040 | |a DE-627 |b ger |c DE-627 |e rda | ||
| 041 | |a eng | ||
| 084 | |a 33 |2 sdnb | ||
| 100 | 1 | |a Neves, Joana |e VerfasserIn |0 (DE-588)1139203711 |0 (DE-627)89695336X |0 (DE-576)493014314 |4 aut | |
| 245 | 1 | 0 | |a Disruption of the Hepcidin/Ferroportin regulatory system causes pulmonary iron overload and restrictive lung disease |c Joana Neves, Dominik Leitz, Simone Kraut, Christina Brandenberger, Raman Agrawal, Norbert Weissmann, Christian Mühlfeld, Marcus A. Mall, Sandro Altamura, Martina U. Muckenthaler |
| 246 | 3 | 0 | |a Hepcidin Ferroportin |
| 264 | 1 | |c 29 April 2017 | |
| 300 | |a 10 | ||
| 336 | |a Text |b txt |2 rdacontent | ||
| 337 | |a Computermedien |b c |2 rdamedia | ||
| 338 | |a Online-Ressource |b cr |2 rdacarrier | ||
| 500 | |a Gesehen am 04.09.2017 | ||
| 520 | |a 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. | ||
| 650 | 4 | |a Ferroportin | |
| 650 | 4 | |a Hepcidin resistance | |
| 650 | 4 | |a Hereditary hemochromatosis | |
| 650 | 4 | |a Iron Overload | |
| 650 | 4 | |a Restrictive lung disease | |
| 700 | 1 | |a Leitz, Dominik |d 1988- |e VerfasserIn |0 (DE-588)1139204629 |0 (DE-627)896955400 |0 (DE-576)493016244 |4 aut | |
| 700 | 1 | |a Agrawal, Raman |e VerfasserIn |0 (DE-588)1067643133 |0 (DE-627)819049689 |0 (DE-576)426814290 |4 aut | |
| 700 | 1 | |a Mall, Marcus A. |d 1975- |e VerfasserIn |0 (DE-588)115507795 |0 (DE-627)691370532 |0 (DE-576)28992152X |4 aut | |
| 700 | 1 | |a Altamura, Sandro |e VerfasserIn |0 (DE-588)1054822247 |0 (DE-627)792338618 |0 (DE-576)410537683 |4 aut | |
| 700 | 1 | |a Muckenthaler, Martina |e VerfasserIn |0 (DE-588)1046124595 |0 (DE-627)775766496 |0 (DE-576)173146813 |4 aut | |
| 773 | 0 | 8 | |i Enthalten in |t EBioMedicine |d Amsterdam [u.a.] : Elsevier, 2014 |g 20(2017), Seite 230-239 |h Online-Ressource |w (DE-627)802540074 |w (DE-600)2799017-5 |w (DE-576)416965725 |x 2352-3964 |7 nnas |a Disruption of the Hepcidin/Ferroportin regulatory system causes pulmonary iron overload and restrictive lung disease |
| 773 | 1 | 8 | |g volume:20 |g year:2017 |g pages:230-239 |g extent:10 |a Disruption of the Hepcidin/Ferroportin regulatory system causes pulmonary iron overload and restrictive lung disease |
| 856 | 4 | 0 | |u http://dx.doi.org/10.1016/j.ebiom.2017.04.036 |x Verlag |x Resolving-System |z kostenfrei |3 Volltext |
| 856 | 4 | 0 | |u http://www.sciencedirect.com/science/article/pii/S2352396417301913 |x Verlag |z kostenfrei |3 Volltext |
| 951 | |a AR | ||
| 992 | |a 20170904 | ||
| 993 | |a Article | ||
| 994 | |a 2017 | ||
| 998 | |g 1046124595 |a Muckenthaler, Martina |m 1046124595:Muckenthaler, Martina |d 910000 |d 910500 |d 700000 |d 718000 |e 910000PM1046124595 |e 910500PM1046124595 |e 700000PM1046124595 |e 718000PM1046124595 |k 0/910000/ |k 1/910000/910500/ |k 0/700000/ |k 1/700000/718000/ |p 10 |y j | ||
| 998 | |g 1054822247 |a Altamura, Sandro |m 1054822247:Altamura, Sandro |d 910000 |d 910500 |e 910000PA1054822247 |e 910500PA1054822247 |k 0/910000/ |k 1/910000/910500/ |p 9 | ||
| 998 | |g 115507795 |a Mall, Marcus A. |m 115507795:Mall, Marcus A. |d 910000 |d 910500 |e 910000PM115507795 |e 910500PM115507795 |k 0/910000/ |k 1/910000/910500/ |p 8 | ||
| 998 | |g 1067643133 |a Agrawal, Raman |m 1067643133:Agrawal, Raman |d 910000 |d 910500 |e 910000PA1067643133 |e 910500PA1067643133 |k 0/910000/ |k 1/910000/910500/ |p 5 | ||
| 998 | |g 1139204629 |a Leitz, Dominik |m 1139204629:Leitz, Dominik |d 910000 |d 910500 |e 910000PL1139204629 |e 910500PL1139204629 |k 0/910000/ |k 1/910000/910500/ |p 2 | ||
| 998 | |g 1139203711 |a Neves, Joana |m 1139203711:Neves, Joana |d 50000 |e 50000PN1139203711 |k 0/50000/ |p 1 |x j | ||
| 999 | |a KXP-PPN1563016567 |e 2979309303 | ||
| BIB | |a Y | ||
| SER | |a journal | ||
| JSO | |a {"name":{"displayForm":["Joana Neves, Dominik Leitz, Simone Kraut, Christina Brandenberger, Raman Agrawal, Norbert Weissmann, Christian Mühlfeld, Marcus A. Mall, Sandro Altamura, Martina U. Muckenthaler"]},"id":{"eki":["1563016567"],"doi":["10.1016/j.ebiom.2017.04.036"]},"origin":[{"dateIssuedDisp":"29 April 2017","dateIssuedKey":"2017"}],"relHost":[{"id":{"eki":["802540074"],"zdb":["2799017-5"],"issn":["2352-3964"]},"origin":[{"publisherPlace":"Amsterdam [u.a.]","dateIssuedDisp":"2014-","dateIssuedKey":"2014","publisher":"Elsevier"}],"physDesc":[{"extent":"Online-Ressource"}],"title":[{"title":"EBioMedicine","title_sort":"EBioMedicine"}],"language":["eng"],"recId":"802540074","note":["Gesehen am 28.05.2020"],"type":{"bibl":"periodical","media":"Online-Ressource"},"disp":"Disruption of the Hepcidin/Ferroportin regulatory system causes pulmonary iron overload and restrictive lung diseaseEBioMedicine","part":{"extent":"10","volume":"20","text":"20(2017), Seite 230-239","pages":"230-239","year":"2017"},"pubHistory":["Volume 1 (2014)-"]}],"physDesc":[{"extent":"10 S."}],"person":[{"family":"Neves","given":"Joana","display":"Neves, Joana","roleDisplay":"VerfasserIn","role":"aut"},{"display":"Leitz, Dominik","roleDisplay":"VerfasserIn","role":"aut","family":"Leitz","given":"Dominik"},{"given":"Raman","family":"Agrawal","role":"aut","roleDisplay":"VerfasserIn","display":"Agrawal, Raman"},{"given":"Marcus A.","family":"Mall","role":"aut","roleDisplay":"VerfasserIn","display":"Mall, Marcus A."},{"role":"aut","display":"Altamura, Sandro","roleDisplay":"VerfasserIn","given":"Sandro","family":"Altamura"},{"role":"aut","roleDisplay":"VerfasserIn","display":"Muckenthaler, Martina","given":"Martina","family":"Muckenthaler"}],"title":[{"title_sort":"Disruption of the Hepcidin/Ferroportin regulatory system causes pulmonary iron overload and restrictive lung disease","title":"Disruption of the Hepcidin/Ferroportin regulatory system causes pulmonary iron overload and restrictive lung disease"}],"language":["eng"],"recId":"1563016567","note":["Gesehen am 04.09.2017"],"type":{"bibl":"article-journal","media":"Online-Ressource"}} | ||
| SRT | |a NEVESJOANADISRUPTION2920 | ||