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Blood, 15 January 2007, Vol. 109, No. 2, pp. 811-818. Prepublished online as a Blood First Edition Paper on September 26, 2006; DOI 10.1182/blood-2006-04-014142. This Article Full Text (PDF) All Versions of this Article: blood-2006-04-014142v1 109/2/811    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 CrossRef Citing Articles via Google Scholar Google Scholar Articles by Lyoumi, S. Articles by Puy, H. Search for Related Content PubMed PubMed Citation Articles by Lyoumi, S. Articles by Puy, H. Social Bookmarking                   What's this?  Previous Article  |  Next Article  Submitted April 3, 2006 Accepted August 11, 2006 Increased plasma transferrin, altered body iron distribution and microcytic hypochromic anemia in ferrochelatase deficient mice Said Lyoumi, Marie Abitbol, Valerie Andrieu, Dominique Henin, Elodie Robert, Caroline Schmitt, Laurent Gouya, Hubert de Verneuil, Jean-Charles Deybach, Xavier Montagutelli, Carole Beaumont*, and Herve Puy INSERM U773, Centre de Recherche Medicale Bichat Beaujon Institut Pasteur, Unite de Genetique des Mammiferes, 75015 Paris, France AP-HP, Hopital Bichat, Laboratoire d'hematologie, 75018 Paris, France AP-HP, Hopital Bichat, Laboratoire d'anatomo-pathologie, 75018 Paris, France INSERM E0217, 33076 Bordeaux, France * Corresponding author; email: beaumont{at}bichat.inserm.fr. Patients with deficiency in ferrochelatase (FECH), the last enzyme of the heme biosynthetic pathway, present painful skin photosensitivity due to the excessive production of protoporphyrin IX (PPIX) by erythrocytes. Controversial results have been reported on both hematological and iron status of these patients with erythropoietic protoporphyria (EPP). We thoroughly explored these parameters in Fechm1Pas mutant mice of three different genetic backgrounds. FECH deficiency induces microcytic hypochromic anemia with absence of ring sideroblasts, little or no hemolysis and no erythroid hyperplasia. Serum iron, ferritin, hepcidin mRNA and Dcytb levels are normal. Homozygous Fechm1Pas mutant have no tissue iron deficiency but show a clear-cut redistribution of iron stores from peripheral tissues to the spleen, with a concomitant 2-3 fold increase in transferrin expression, both at the mRNA and protein levels. Erythrocyte PPIX levels strongly correlated to serum transferrin levels. In Fechm1Pas mutant mice and in contrast to iron-deficiency anemia, transferrin receptor expression in bone marrow erythroid cells was normal, at all stages of differentiation. Based on these observations, we suggest that oral iron therapy may not the therapy of choice in EPP patients and that the PPIX- liver transferrin pathway plays a role in the orchestration of iron distribution between peripheral iron stores, the spleen and the bone marrow. CiteULike    Connotea    Del.icio.us    Digg    Reddit    Technorati    What's this?

	Copyright © 2006 by American Society of Hematology         Online ISSN: 1528-0020