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

Hfe acts in hepatocytes to prevent hemochromatosis.

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1
Department of Pediatric Oncology, Hematology and Immunology, University Hospital of Heidelberg, 69120 Heidelberg, Germany.

Abstract

Hereditary hemochromatosis (HH) is a prevalent, potentially fatal disorder of iron metabolism hallmarked by intestinal hyperabsorption of iron, hyperferremia, and hepatic iron overload. 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.

PMID:
18249176
DOI:
10.1016/j.cmet.2007.11.014
[Indexed for MEDLINE]
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