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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 (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 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 Related Article in Blood Online Social Bookmarking                   What's this?  Previous Article  |  Next Article  Submitted October 6, 2004 Accepted October 27, 2004 Iron trafficking in the mitochondrion: novel pathways revealed by disease Ian Napier, Prem Ponka, and Des R Richardson* Iron Metabolism and Chelation Program, Children's Cancer Institute Australia for Medical Research, Sydney, New South Wales, Australia Lady Davis Institute for Medical Research, Montreal, Quebec, Canada * Corresponding author; email: d.richardson{at}ccia.org.au. 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 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 not only vital for heme synthesis, but playing a critical role in the genesis of [Fe-S] clusters. Studies examining the disease, Friedreichs ataxia, have suggested that a mutation in the gene encoding frataxin leads to mitochondrial Fe-loading. Apart from these findings, the recent discovery of mitochondrial ferritin that may store Fe in ring sideroblasts, may 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. 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