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Cell-specific location of Hfe: It is the 'cyte that matters

✍ Scribed by Darrell H. G. Crawford; Linda M. Fletcher; Gregory J. Anderson


Publisher
John Wiley and Sons
Year
2008
Tongue
English
Weight
70 KB
Volume
48
Category
Article
ISSN
0270-9139

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✦ Synopsis


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 1 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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