Format

Send to

Choose Destination
See comment in PubMed Commons below
J Clin Invest. 2005 Aug;115(8):2180-6.

Hemojuvelin is essential for dietary iron sensing, and its mutation leads to severe iron overload.

Author information

  • 1Biozentrum, Department of Cell Biology, University of Basel, and Friedrich Miescher Institute, Basel, Switzerland.

Abstract

Iron homeostasis plays a critical role in many physiological processes, notably synthesis of heme proteins. Dietary iron sensing and inflammation converge in the control of iron absorption and retention by regulating the expression of hepcidin, a regulator of the iron exporter ferroportin. Human mutations in the glycosylphosphatidylinositol-anchored protein hemojuvelin (HJV; also known as RGMc and HFE2) cause juvenile hemochromatosis, a severe iron overload disease, but the way in which HJV intersects with the iron regulatory network has been unclear. Here we show that, within the liver, mouse Hjv is selectively expressed by periportal hepatocytes and also that Hjv-mutant mice exhibit iron overload as well as a dramatic decrease in hepcidin expression. Our findings define a key role for Hjv in dietary iron sensing and also reveal that cytokine-induced inflammation regulates hepcidin expression through an Hjv-independent pathway.

Comment in

PMID:
16075058
PMCID:
PMC1180556
DOI:
10.1172/JCI25683
[PubMed - indexed for MEDLINE]
Free PMC Article
PubMed Commons home

PubMed Commons

0 comments
How to join PubMed Commons

    Supplemental Content

    Full text links

    Icon for Journal of Clinical Investigation Icon for PubMed Central
    Loading ...
    Support Center