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Blood, 15 November 2005, Vol. 106, No. 10, pp. 3625-3631. Prepublished online as a Blood First Edition Paper on June 30, 2005; DOI 10.1182/blood-2005-01-0379. This Article Full Text Full Text (PDF) All Versions of this Article: 2005-01-0379v1 106/10/3625    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 Ohgami, R. S. Articles by Fleming, M. D. Search for Related Content PubMed PubMed Citation Articles by Ohgami, R. S. Articles by Fleming, M. D. Related Collections Hematopoiesis and Stem Cells Red Cells Social Bookmarking                   What's this?  Previous Article  |  Table of Contents  |  Next Article  RED CELLS nm1054: a spontaneous, recessive, hypochromic, microcytic anemia mutation in the mouse Robert S. Ohgami, Dean R. Campagna, Brendan Antiochos, Emily B. Wood, John J. Sharp, Jane E. Barker, and Mark D. Fleming From the Department of Pathology, Children's Hospital and Harvard Medical School, Boston, MA; the Jackson Laboratory, Bar Harbor, ME; and the Department of Human and Molecular Genetics, Baylor College of Medicine, Houston, TX. Hypochromic, microcytic anemias are typically the result of inadequate hemoglobin production because of globin defects or iron deficiency. Here, we describe the phenotypic characteristics and pathogenesis of a new recessive, hypochromic, microcytic anemia mouse mutant, nm1054. Although the mutation nm1054 is pleiotropic, also resulting in sparse hair, male infertility, failure to thrive, and hydrocephaly, the anemia is the focus of this study. Hematologic analysis reveals a moderately severe, congenital, hypochromic, microcytic anemia, with an elevated red cell zinc protoporphyrin, consistent with functional erythroid iron deficiency. However, serum and tissue iron analyses show that nm1054 animals are not systemically iron deficient. From hematopoietic stem cell transplantation and iron uptake studies in nm1054 reticulocytes, we provide evidence that the nm1054 anemia is due to an intrinsic hematopoietic defect resulting in inefficient transferrin-dependent iron uptake by erythroid precursors. Linkage studies demonstrate that nm1054 maps to a genetic locus not previously implicated in microcytic anemia or iron phenotypes. CiteULike    Connotea    Del.icio.us    Digg    Reddit    Technorati    What's this? This article has been cited by other articles: A. K. Sendamarai, R. S. Ohgami, M. D. Fleming, and C. M. Lawrence Structure of the membrane proximal oxidoreductase domain of human Steap3, the dominant ferrireductase of the erythroid transferrin cycle PNAS, May 27, 2008; 105(21): 7410 - 7415. [Abstract] [Full Text] [PDF] L. Lee, D. R. Campagna, J. L. Pinkus, H. Mulhern, T. A. Wyatt, J. H. Sisson, J. A. Pavlik, G. S. Pinkus, and M. D. Fleming Primary Ciliary Dyskinesia in Mice Lacking the Novel Ciliary Protein Pcdp1 Mol. Cell. Biol., February 1, 2008; 28(3): 949 - 957. [Abstract] [Full Text] [PDF] C. Pondarre, D. R. Campagna, B. Antiochos, L. Sikorski, H. Mulhern, and M. D. Fleming Abcb7, the gene responsible for X-linked sideroblastic anemia with ataxia, is essential for hematopoiesis Blood, April 15, 2007; 109(8): 3567 - 3569. [Abstract] [Full Text] [PDF] R. S. Ohgami, D. R. Campagna, A. McDonald, and M. D. Fleming The Steap proteins are metalloreductases Blood, August 15, 2006; 108(4): 1388 - 1394. [Abstract] [Full Text] [PDF]

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