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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 (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 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. Social Bookmarking                   What's this? Submitted July 5, 2002 Accepted October 4, 2002 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 Department of Hematology, University of Pavia and IRCCS Policlinico S. Matteo, Pavia, Italy Department of Internal Medicine, University of Pavia and IRCCS Policlinico S. Matteo, Pavia, Italy DIBIT IRCCS S. Raffaele Hospital, Protein Engineering Unit, Milan, Italy Department of Pediatrics and Biomedical Technology, University of Brescia School of Medicine, Brescia, Italy Department of Biochemistry, Tufts University School of Medicine, Boston, MA, USA * Corresponding author; email: mario.cazzola{at}unipv.it. The sideroblastic anemias are characterized by ring sideroblasts, i.e., 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 of MtF using immunocytochemical methods. We also studied 11 normal 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 a diffuse cytoplasmic positivity for HF, but very few were positive for MtF (0-10%), and 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, the positivity regularly presenting granules ringing the nucleus. Double immunocytochemical staining confirmed the different cellular distribution of HF and MtF. There was a highly significant relationship between percentage of MtF-positive erythroblasts and that of ring sideroblasts (Spearman R = 0.90, P < 0.0001). RT-PCR studies of circulating reticulocyte RNA demonstrated the presence of MtF mRNA in two 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 mitochondrial ferritin, and that this latter might be a specific marker of sideroblastic anemia. CiteULike    Connotea    Del.icio.us    Digg    Reddit    Technorati    What's this? This article has been cited by other articles: L. Malcovati and M. Cazzola Myelodysplastic/myeloproliferative disorders Haematologica, January 1, 2008; 93(1): 4 - 6. [Full Text] [PDF] P. Santambrogio, G. Biasiotto, F. Sanvito, S. Olivieri, P. Arosio, and S. 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