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However, little is known concerning the regulation of iron uptake by the mitochondrion and how this is coordinated with iron metabolism in the cytosol and other organelles (e.g., lysosomes). In this article, we discuss the burgeoning field of mitochondrial iron metabolism and trafficking that has recently been stimulated by the discovery of proteins involved in mitochondrial iron storage (mitochondrial ferritin) and transport (mitoferrin-1 and -2). In addition, recent work examining mitochondrial diseases (e.g., Friedreich's ataxia) has established that communication exists between iron metabolism in the mitochondrion and the cytosol. This finding has revealed the ability of the mitochondrion to modulate whole-cell iron-processing to satisfy its own requirements for the crucial processes of heme and ISC synthesis. Knowledge of mitochondrial iron-processing pathways and the interaction between organelles and the cytosol could revolutionize the investigation of iron metabolism.<\/jats:p>","DOI":"10.1073\/pnas.0912925107","type":"journal-article","created":{"date-parts":[[2010,5,21]],"date-time":"2010-05-21T22:18:59Z","timestamp":1274480339000},"page":"10775-10782","update-policy":"https:\/\/doi.org\/10.1073\/pnas.cm10313","source":"Crossref","is-referenced-by-count":480,"title":["Mitochondrial iron trafficking and the integration of iron metabolism between the mitochondrion and cytosol"],"prefix":"10.1073","volume":"107","author":[{"given":"Des R.","family":"Richardson","sequence":"first","affiliation":[{"name":"Iron Metabolism and Chelation Program, Discipline of Pathology, University of Sydney, NSW 2006, Australia;"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Darius J. 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