Mitochondrial iron trafficking and the integration of iron metabolism between the mitochondrion and cytosol
Open accessAbstract
The mitochondrion is well known for its key role in energy transduction. However, it is less well appreciated that it is also a focal point of iron metabolism. Iron is needed not only for heme and iron sulfur cluster (ISC)-containing proteins involved in electron transport and oxidative phosphorylation, but also for a wide variety of cytoplasmic and nuclear functions, including DNA synthesis. The mitochondrial pathways involved in the generation of both heme and ISCs have been characterized to some extent. 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.
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Imbalanced iron levels in the cell can lead to dysfunctional mitochondria, increased oxidative damage, and increased toxicity.
“Imbalanced iron levels in the cell can lead to dysfunctional mitochondria, increased oxidative damage and increased toxicity (Richardson et al., 2010; Volani et al., 2017).”
From The Role of Iron Regulation in Immunometabolism and Immune-Related Disease (2019) · cites this paper as Richardson et al., 2010
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Frataxin, a molecule of mystery: trading stability for function in its iron-binding site
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Weiss, G., Ganz, T., & Goodnough, L. T. (2019) · Blood · doi:10.1182/blood-2018-06-856500 · Cited by 1,219 · see citing works
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- Authors
- Richardson, D. R., Lane, D. J. R., Becker, E. M., Huang, M. L.-H., Whitnall, M., Rahmanto, Y. S., Sheftel, A. D., & Ponka, P.
- Affiliations
- The University of Sydney (AU)
- Philipps University of Marburg (DE)
- Jewish General Hospital (CA)
- Venue
- Proceedings of the National Academy of Sciences
- Publisher
- National Academy of Sciences
- Published
- 2010-05-21
- Volume
- 107
- Issue
- 24
- Pages
- 10775-10782
- ISSN
- 0027-8424 (ISSN-L)
- 1091-6490
- PMC
- PMC2890738
- OpenAlex
- W2018411741
- Cited by
- 536
- References
- 108
- Citations by year
- 2026: 28 · 2025: 28 · 2024: 32 · 2023: 29 · 2022: 29 · 2021: 43 · 2020: 40 · 2019: 39 · 2018: 34 · 2017: 19 · 2016: 30 · 2015: 31 · 2014: 37 · 2013: 39 · 2012: 38
- FWCI
- 5.91
- Type
- journal article
- Language
- en
- Open access
- green
- europepmc.org
- Locations
- Proceedings of the National Academy of Sciences (publishedVersion)
- Europe PMC (PubMed Central) (publishedVersion)
- PubMed Central (publishedVersion)
- PubMed (publishedVersion)
- Funders
- Friedreich's Ataxia Research Alliance
- Muscular Dystrophy Association
- Cancer Institute NSW
- Canadian Institutes of Health Research
- Medical Research Council
- National Health and Medical Research Council
- Topics
- Health Sciences › Medicine › Hematology › Iron Metabolism and Disorders
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Molecular Biology › Mitochondrial Function and Pathology
- Life Sciences › Neuroscience › Neurology › Neurological diseases and metabolism
- Keywords
- mitochondrial iron metabolism, iron trafficking, mitochondrial ferritin, heme biosynthesis, iron-sulfur clusters, Friedreich's ataxia, mitochondrial disease, iron absorption, lysosomes, DNA synthesis
- MeSH terms
- Frataxin, Anemia, Sideroblastic, Animals, Biological Transport, Active, Cytosol, Friedreich Ataxia, Heme, Homeostasis, Humans, Iron, Iron-Sulfur Proteins, Mitochondria, Models, Biological, Receptors, Transferrin, Transferrin, Iron-Binding Proteins
About these checks
Registry, retraction, Cites and statistics results are computed, not judged, with one exception: a printed reference that no index lists is read once by a model before it counts as a citation error. Claims are extracted by a model and kept only when their quote appears verbatim in the text; each cited source is then checked, with a model’s help, for whether it says what the claim relies on. Those verdicts can be wrong — read the claim’s quote and the cited source before relying on one.
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