SEARCH:   Advanced

Blood, 1 April 2004, Vol. 103, No. 7, pp. 2847-2849. Prepublished online as a Blood First Edition Paper on December 4, 2003; DOI 10.1182/blood-2003-09-3300. This Article Full Text (PDF) All Versions of this Article: 2003-09-3300v1 103/7/2847    most recent Alert me when this article is cited Alert me if a correction is posted 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 Miranda, C. J Articles by Santos, M. M Search for Related Content PubMed PubMed Citation Articles by Miranda, C. J Articles by Santos, M. M Social Bookmarking                   What's this? Submitted September 25, 2003 Accepted November 21, 2003 Contributions of 2-microglobulin-dependent molecules and lymphocytes to iron regulation: insights from HfeRag1-/- and 2mRag1-/- double-knockout mice Carlos J Miranda, Hortence Makui, Nancy C Andrews, and Manuela M Santos* Centre de Recherche, Centre Hospitalier de l'Universite de Montreal, University of Montreal, Montreal, PQ, Canada Department of Pediatrics, Howard Hughes Medical Institute, Boston, MA, USA UnIGENe, Instituto de Biologia Molecular e Celular, Porto, Portugal * Corresponding author; email: msantos{at}istar.ca. Genetic causes of hereditary hemochromatosis (HH) include mutations in the HFE gene, coding for a 2-microglobulin (2m)-associated major histocompatibility complex class I-like (MHC-I) protein. However, iron accumulation in HH patients can be highly variable. Previously, analysis of 2mRag1-/- double-deficient mice, lacking all 2m-dependent molecules and lymphocytes, demonstrated increased iron accumulation in the pancreas and heart compared to 2m single-knockout mice. To evaluate whether the observed phenotype in 2mRag1-/- mice was due solely to the absence of Hfe or to other 2m-dependent molecules, we generated HfeRag1-/- double-deficient mice. Our studies revealed that introduction of Rag1-deficiency in Hfe-knockout mice leads to heightened iron overload, mainly in the liver, while the heart and pancreas are relatively spared compared to 2mRag-/- mice. These results suggest that other 2m-interacting protein(s) may be involved in iron regulation, and that in the absence of functional HFE molecules, lymphocyte numbers may influence iron overload severity. CiteULike    Connotea    Del.icio.us    Digg    Reddit    Technorati    What's this? This article has been cited by other articles: M. Constante, D. Wang, V.-A. Raymond, M. Bilodeau, and M. M. Santos Repression of Repulsive Guidance Molecule C during Inflammation Is Independent of Hfe and Involves Tumor Necrosis Factor-{alpha} Am. J. Pathol., February 1, 2007; 170(2): 497 - 504. [Abstract] [Full Text] [PDF] C. Chaudhury, J. Kim, S. Mehnaz, M. A. Wani, T. M. Oberyszyn, C. L. Bronson, S. Mohanty, W. L. Hayton, J. M. Robinson, and C. L. Anderson Accelerated Transferrin Degradation in HFE-Deficient Mice Is Associated with Increased Transferrin Saturation J. Nutr., December 1, 2006; 136(12): 2993 - 2998. [Abstract] [Full Text] [PDF] M. Constante, W. Jiang, D. Wang, V.-A. Raymond, M. Bilodeau, and M. M. Santos Distinct requirements for Hfe in basal and induced hepcidin levels in iron overload and inflammation Am J Physiol Gastrointest Liver Physiol, August 1, 2006; 291(2): G229 - G237. [Abstract] [Full Text] [PDF] H. Makui, R. J. Soares, W. Jiang, M. Constante, and M. M. Santos Contribution of Hfe expression in macrophages to the regulation of hepatic hepcidin levels and iron loading Blood, September 15, 2005; 106(6): 2189 - 2195. [Abstract] [Full Text] [PDF] P. S. Rohrlich, N. Fazilleau, F. Ginhoux, H. Firat, F. Michel, M. Cochet, N. Laham, M. P. Roth, S. Pascolo, F. Nato, et al. From The Cover: Direct recognition by {alpha}{beta} cytolytic T cells of Hfe, a MHC class Ib molecule without antigen-presenting function PNAS, September 6, 2005; 102(36): 12855 - 12860. [Abstract] [Full Text] [PDF] S. Rivera, L. Liu, E. Nemeth, V. Gabayan, O. E. Sorensen, and T. Ganz Hepcidin excess induces the sequestration of iron and exacerbates tumor-associated anemia Blood, February 15, 2005; 105(4): 1797 - 1802. [Abstract] [Full Text] [PDF]

	Copyright © 2003 by American Society of Hematology         Online ISSN: 1528-0020