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Citations to this article

A mouse model of juvenile hemochromatosis
Franklin W. Huang, … , Mark D. Fleming, Nancy C. Andrews
Franklin W. Huang, … , Mark D. Fleming, Nancy C. Andrews
Published August 1, 2005
Citation Information: J Clin Invest. 2005;115(8):2187-2191. https://doi.org/10.1172/JCI25049.
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Research Article Genetics Article has an altmetric score of 12

A mouse model of juvenile hemochromatosis

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Abstract

Hereditary hemochromatosis is an iron-overload disorder resulting from mutations in proteins presumed to be involved in the maintenance of iron homeostasis. Mutations in hemojuvelin (HJV) cause severe, early-onset juvenile hemochromatosis. The normal function of HJV is unknown. Juvenile hemochromatosis patients have decreased urinary levels of hepcidin, a peptide hormone that binds to the cellular iron exporter ferroportin, causing its internalization and degradation. We have disrupted the murine Hjv gene and shown that Hjv–/– mice have markedly increased iron deposition in liver, pancreas, and heart but decreased iron levels in tissue macrophages. Hepcidin mRNA expression was decreased in Hjv–/– mice. Accordingly, ferroportin expression detected by immunohistochemistry was markedly increased in both intestinal epithelial cells and macrophages. We propose that excess, unregulated ferroportin activity in these cell types leads to the increased intestinal iron absorption and plasma iron levels characteristic of the juvenile hemochromatosis phenotype.

Authors

Franklin W. Huang, Jack L. Pinkus, Geraldine S. Pinkus, Mark D. Fleming, Nancy C. Andrews

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Total citations by year

Year: 2025 2024 2023 2022 2021 2020 2019 2018 2017 2016 2015 2014 2013 2012 2011 2010 2009 2008 2007 2006 2005 Total
Citations: 3 5 6 3 9 5 6 11 10 10 8 12 10 12 12 18 15 7 8 9 1 180
Citation information
This citation data is accumulated from CrossRef, which receives citation information from participating publishers, including this journal. Not all publishers participate in CrossRef, so this information is not comprehensive. Additionally, data may not reflect the most current citations to this article, and the data may differ from citation information available from other sources (for example, Google Scholar, Web of Science, and Scopus).

Citations to this article in year 2007 (8)

Title and authors Publication Year
Modulation of Bone Morphogenetic Protein Signaling in vivo Regulates Systemic Iron Balance
Jodie L. Babitt, Franklin W. Huang, Yin Xia, Yisrael Sidis, Nancy C. Andrews, Herbert Y. Lin
Journal of Clinical Investigation 2007
Regulation of iron homeostasis by the hypoxia inducible transcription factor-1 (HIF-1)
Carole Peyssonnaux, Annelies S. Zinkernagel, Reto A. Schuepbach, Erinn Rankin, Sophie Vaulont, Volker H. Haase, Victor Nizet and Randall S. Johnson
Journal of Clinical Investigation 2007
The relevance of the intestinal crypt and enterocyte in regulating iron absorption
PS Oates
Pflügers Archiv - European Journal of Physiology 2007
Repression of repulsive guidance molecule C during inflammation is independent of Hfe and involves tumor necrosis factor-alpha
M Constante, D Wang, VA Raymond, M Bilodeau, MM Santos
The American Journal of Pathology 2007
Ineffective erythropoiesis in beta-thalassemia is characterized by increased iron absorption mediated by down-regulation of hepcidin and up-regulation of ferroportin
S Gardenghi, MF Marongiu, P Ramos, E Guy, L Breda, A Chadburn, YF Liu, N Amariglio, G Rechavi, EA Rachmilewitz, W Breuer, ZI Cabantchik, DM Wrighting, NC Andrews, M Sousa, PJ Giardina, RW Grady, S Rivella
Blood 2007
Non-HFE haemochromatosis.
Wallace DF, Subramaniam VN
World Journal of Gastroenterology 2007
Liver iron transport.
Graham RM, Chua AC, Herbison CE, Olynyk JK, Trinder D
World Journal of Gastroenterology 2007
Liver-gut axis in the regulation of iron homeostasis.
Darshan D, Anderson GJ
World Journal of Gastroenterology 2007

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