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Prepublished online as a Blood First Edition Paper on October 24, 2002; DOI 10.1182/blood-2002-07-2006. This Article Full Text Full Text (PDF) All Versions of this Article: 2002-07-2006v1 101/5/1996    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 Cazzola, M. Articles by Arosio, P. Search for Related Content PubMed PubMed Citation Articles by Cazzola, M. Articles by Arosio, P. Related Collections Neoplasia Red Cells Social Bookmarking                   What's this?  Previous Article  |  Table of Contents  |  Next Article  Blood, 1 March 2003, Vol. 101, No. 5, pp. 1996-2000 RED CELLS Mitochondrial ferritin expression in erythroid cells from patients with sideroblastic anemia Mario Cazzola, Rosangela Invernizzi, Gaetano Bergamaschi, Sonia Levi, Barbara Corsi, Erica Travaglino, Valeria Rolandi, Giorgio Biasiotto, Jim Drysdale, and Paolo Arosio From the Department of Hematology and Department of Internal Medicine and Medical Therapy, University of Pavia Medical School and Istituto di Ricovero e Cura a Carattere Scientifico (IRCCS) Policlinico S Matteo, Pavia, Italy; Protein Engineering Unit, DIBIT IRCCS S Raffaele Hospital, Milan, Italy; Department of Pediatrics and Biomedical Technology, University of Brescia School of Medicine, Brescia, Italy; and Department of Biochemistry, Tufts University School of Medicine, Boston, MA. The sideroblastic anemias are characterized by ring sideroblasts, that is, red cell precursors with mitochondrial iron accumulation. We therefore studied the expression of mitochondrial ferritin (MtF) in these conditions. Erythroid cells from 13 patients with refractory anemia with ring sideroblasts (RARS) and 3 patients with X-linked sideroblastic anemia (XLSA) were analyzed for the distribution of cytoplasmic H ferritin (HF) and MtF using immunocytochemical methods. We also studied 11 healthy controls, 5 patients with refractory anemia without ring sideroblasts (RA), and 7 patients with RA with excess of blasts (RAEB). About one fourth of normal immature red cells, mostly proerythroblasts and basophilic erythroblasts, showed diffuse cytoplasmic positivity for HF, but very few were positive for MtF (0%-10%). Similar patterns were found in anemic patients without ring sideroblasts. In contrast, many erythroblasts from patients with sideroblastic anemia (82%-90% in XLSA and 36%-84% in RARS) were positive for MtF, which regularly appeared as granules ringing the nucleus. Double immunocytochemical staining confirmed the different cellular distribution of HF and MtF. There was a highly significant relationship between the percentage of MtF+ erythroblasts and that of ring sideroblasts (Spearman R = 0.90; P < .0001). Reverse transcription-polymerase chain reaction studies demonstrated the presence of MtF mRNA in circulating reticulocytes of 2 patients with XLSA but not in controls. These findings suggest that most of the iron deposited in perinuclear mitochondria of ring sideroblasts is present in the form of MtF and that this latter might be a specific marker of sideroblastic anemia. © 2003 by The American Society of Hematology.   CiteULike    Connotea    Del.icio.us    Digg    Reddit    Technorati    What's this? This article has been cited by other articles: L. 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