Hfe acts in hepatocytes to prevent hemochromatosis
|Authors:||Vujić Spasić M, Kiss J, Herrmann T, Galy B, Martinache S, Stolte J, Gröne HJ, Stremmel W, Hentze MW, Muckenthaler MU.|
|CellNetworks People:||Muckenthaler Martina, Hentze Matthias|
|Journal:||Cell Metab. 2008 Feb;7(2):173-8.|
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.