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Blood, 1 March 2005, Vol. 105, No. 5, pp. 1867-1874. Prepublished online as a Blood First Edition Paper on November 4, 2004; DOI 10.1182/blood-2004-10-3856. This Article Full Text Full Text (PDF) All Versions of this Article: 2004-10-3856v1 105/5/1867    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 Napier, I. Articles by Richardson, D. R. Search for Related Content PubMed PubMed Citation Articles by Napier, I. Articles by Richardson, D. R. Related Collections Free Research Articles Review Articles Red Cells Related Article in Blood Online Social Bookmarking                   What's this?  Previous Article  |  Table of Contents  |  Next Article  REVIEW ARTICLES Iron trafficking in the mitochondrion: novel pathways revealed by disease Ian Napier, Prem Ponka, and Des R. Richardson From the Children's Cancer Institute Australia for Medical Research, Iron Metabolism and Chelation Program, Randwick, Sydney, New South Wales, Australia; and Lady Davis Institute for Medical Research, Montreal, Quebec, Canada. It is well known that iron (Fe) is transported to the mitochondrion for heme synthesis. However, only recently has the importance of this organelle for many other facets of Fe metabolism become widely appreciated. Indeed, this was stimulated by the description of human disease states that implicate mitochondrial Fe metabolism. In particular, studies assessing various diseases leading to mitochondrial Fe loading have produced intriguing findings. For instance, the disease X-linked sideroblastic anemia with ataxia (XLSA/A) is due to a mutation in the ATP-binding cassette protein B7 (ABCB7) transporter that is thought to transfer [Fe-S] clusters from the mitochondrion to the cytoplasm. This and numerous other findings suggest the mitochondrion is a dynamo of Fe metabolism, being vital not only for heme synthesis but also for playing a critical role in the genesis of [Fe-S] clusters. Studies examining the disease Friedreich ataxia have suggested that a mutation in the gene encoding frataxin leads to mitochondrial Fe loading. Apart from these findings, the recently discovered mitochondrial ferritin that may store Fe in ring sideroblasts could also regulate the level of Fe needed for heme and [Fe-S] cluster synthesis. In this review, we suggest a model of mitochondrial Fe processing that may account for the pathology observed in these disease states. CiteULike    Connotea    Del.icio.us    Digg    Reddit    Technorati    What's this? Related Article in Blood Online: Cellular iron metabolism: mitochondria in the spotlight Christopher R. Chitambar Blood 2005 105: 1844-1845. [Full Text] [PDF] This article has been cited by other articles: C. K. Lim, D. S. Kalinowski, and D. R. Richardson Protection against Hydrogen Peroxide-Mediated Cytotoxicity in Friedreich's Ataxia Fibroblasts Using Novel Iron Chelators of the 2-Pyridylcarboxaldehyde Isonicotinoyl Hydrazone Class Mol. Pharmacol., July 1, 2008; 74(1): 225 - 235. [Abstract] [Full Text] [PDF] M. A. Burke, R. K. Mutharasan, and H. Ardehali The Sulfonylurea Receptor, an Atypical ATP-Binding Cassette Protein, and Its Regulation of the KATP Channel Circ. Res., February 1, 2008; 102(2): 164 - 176. [Abstract] [Full Text] [PDF] G. 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