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Blood, 15 August 2006, Vol. 108, No. 4, pp. 1402-1405. Prepublished online as a Blood First Edition Paper on March 30, 2006; DOI 10.1182/blood-2006-02-003376. This Article Full Text Full Text (PDF) All Versions of this Article: blood-2006-02-003376v1 108/4/1402    most recent Alert me when this article is cited Alert me if a correction is posted Citation Map Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Download to citation manager Rights and Permissions Citing Articles Citing Articles via HighWire Citing Articles via CrossRef Citing Articles via Google Scholar Google Scholar Articles by Lesbordes-Brion, J.-C. Articles by Vaulont, S. Search for Related Content PubMed PubMed Citation Articles by Lesbordes-Brion, J.-C. Articles by Vaulont, S. Related Collections Red Cells Brief Reports Social Bookmarking                   What's this?  Previous Article  |  Table of Contents  |  Next Article  RED CELLS Brief report Targeted disruption of the hepcidin 1 gene results in severe hemochromatosis Jeanne-Claire Lesbordes-Brion, Lydie Viatte, Myriam Bennoun, Dan-Qing Lou, Guillemette Ramey, Christophe Houbron, Ghislaine Hamard, Axel Kahn, and Sophie Vaulont From the Institut Cochin, Département de Génétique, Développement et Pathologie Moléculaire; Institut National de la Santé et de la Recherche Médicale (INSERM) U567; Centre National de la Recherche Scientifique (CNRS); and Université Paris Descartes, Faculté de Médecine René Descartes, Paris, France. We previously reported that mice made deficient for the transcriptional factor USF2 fail to express hepcidin 1 and hepcidin 2 genes as a consequence of targeted disruption of the Usf2 gene lying just upstream in the locus. These mice developed an iron overload phenotype with excess iron deposition in parenchymal cells and decreased reticuloendothelial iron. At that time, although the role of USF2 was still confounding, we proposed for the first time the role of hepcidin as a negative regulator of iron absorption and iron release from macrophages. Accordingly, we subsequently demonstrated that hyperexpression of hepcidin 1, but not hepcidin 2, resulted in a profound hyposideremic anemia. To analyze the consequences of hepcidin 1 deletion on iron metabolism without any disturbance due to USF2 deficiency, we disrupted the hepcidin 1 gene by targeting almost all the coding region. Confirming our prior results, Hepc1–/– mice developed early and severe multivisceral iron overload, with sparing of the spleen macrophages, and demonstrated increased serum iron and ferritin levels as compared with their controls. CiteULike    Connotea    Del.icio.us    Digg    Reddit    Technorati    What's this? This article has been cited by other articles: D. W. Lee, S. Rajagopalan, A. Siddiq, R. Gwiazda, L. Yang, M. F. Beal, R. R. Ratan, and J. K. Andersen Inhibition of Prolyl Hydroxylase Protects against 1-Methyl-4-phenyl-1,2,3,6-tetrahydropyridine-induced Neurotoxicity: MODEL FOR THE POTENTIAL INVOLVEMENT OF THE HYPOXIA-INDUCIBLE FACTOR PATHWAY IN PARKINSON DISEASE J. Biol. Chem., October 16, 2009; 284(42): 29065 - 29076. [Abstract] [Full Text] [PDF] G. Peslova, J. Petrak, K. Kuzelova, I. Hrdy, P. Halada, P. W. Kuchel, S. Soe-Lin, P. Ponka, R. Sutak, E. Becker, et al. Hepcidin, the hormone of iron metabolism, is bound specifically to {alpha}-2-macroglobulin in blood Blood, June 11, 2009; 113(24): 6225 - 6236. [Abstract] [Full Text] [PDF] L. Kautz, D. Meynard, A. Monnier, V. Darnaud, R. Bouvet, R.-H. Wang, C. Deng, S. Vaulont, J. Mosser, H. Coppin, et al. Iron regulates phosphorylation of Smad1/5/8 and gene expression of Bmp6, Smad7, Id1, and Atoh8 in the mouse liver Blood, August 15, 2008; 112(4): 1503 - 1509. [Abstract] [Full Text] [PDF] N. C. Andrews Forging a field: the golden age of iron biology Blood, July 15, 2008; 112(2): 219 - 230. [Full Text] [PDF] M. D. Fleming The Regulation of Hepcidin and Its Effects on Systemic and Cellular Iron Metabolism Hematology, January 1, 2008; 2008(1): 151 - 158. [Abstract] [Full Text] [PDF]

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