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. 2008 Feb;7(2):173-8.
doi: 10.1016/j.cmet.2007.11.014.

Hfe acts in hepatocytes to prevent hemochromatosis

Maja Vujić Spasić  1 , Judit Kiss, Thomas Herrmann, Bruno Galy, Stefanie Martinache, Jens Stolte, Hermann-Josef Gröne, Wolfgang Stremmel, Matthias W Hentze, Martina U Muckenthaler
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Free article

Hfe acts in hepatocytes to prevent hemochromatosis

Maja Vujić Spasić et al. Cell Metab. 2008 Feb.
Free article

Abstract

Hereditary hemochromatosis (HH) is a prevalent, potentially fatal disorder of iron metabolism hallmarked by intestinal hyperabsorption of iron, hyperferremia, and hepatic iron overload VSports手机版. In both humans and mice, type I HH is associated with mutations in the broadly expressed HFE/Hfe gene. To identify where Hfe acts to prevent HH, we generated mice with tissue-specific Hfe ablations. This work demonstrates that local Hfe expression in hepatocytes serves to maintain physiological iron homeostasis, answering a long-standing question in medicine and explaining earlier clinical observations. .

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