Mitochondrial Mayhem: The Mitochondrion as a Modulator of Iron Metabolism and Its Role in Disease
Abstract
The mitochondrion plays vital roles in various aspects of cellular metabolism, ranging from energy transduction and apoptosis to the synthesis of important molecules such as heme. Mitochondria are also centrally involved in iron metabolism, as exemplified by disruptions in mitochondrial proteins that lead to perturbations in whole-cell iron processing. Recent investigations have identified a host of mitochondrial proteins (e.g., mitochondrial ferritin; mitoferrins 1 and 2; ABCBs 6, 7, and 10; and frataxin) that may play roles in the homeostasis of mitochondrial iron. These mitochondrial proteins appear to participate in one or more processes of iron storage, iron uptake, and heme and iron-sulfur cluster synthesis. In this review, we present and critically discuss the evidence suggesting that the mitochondrion may contribute to the regulation of whole-cell iron metabolism. Further, human diseases that arise from a dysregulation of these mitochondrial molecules reveal the ability of the mitochondrion to communicate with cytosolic iron metabolism to coordinate whole-cell iron processing and to fulfill the high demands of this organelle for iron. This review highlights new advances in understanding iron metabolism in terms of novel molecular players and diseases associated with its dysregulation.
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Fe-S mitochondrial disruptions can cause cytosolic iron depletion.
“Indeed, such Fe-S mitochondrial disruptions can cause cytosolic iron depletion (Huang et al., 2011).”
From The Role of Iron Regulation in Immunometabolism and Immune-Related Disease (2019) · cites this paper as Huang et al., 2011
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- Authors
- Huang, M. L.-H., Lane, D. J. R., & Richardson, D. R.
- Affiliations
- The University of Sydney (AU)
- Venue
- Antioxidants & Redox Signaling
- Publisher
- SAGE Publications
- Published
- 2011-12
- Volume
- 15
- Issue
- 12
- Pages
- 3003-3019
- ISSN
- 1523-0864 (ISSN-L)
- 1557-7716
- OpenAlex
- W2080571868
- Cited by
- 103
- References
- 157
- Citations by year
- 2026: 5 · 2025: 8 · 2024: 8 · 2023: 4 · 2022: 5 · 2021: 8 · 2020: 4 · 2019: 14 · 2018: 8 · 2017: 5 · 2016: 7 · 2015: 4 · 2014: 7 · 2013: 10 · 2012: 6
- FWCI
- 0.98
- Type
- journal article
- Language
- en
- Open access
- closed
- Locations
- Antioxidants and Redox Signaling (publishedVersion)
- PubMed (publishedVersion)
- 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, mitochondrial ferritin, ABCB6, ABCB7, ABCB10, frataxin, heme biosynthesis, iron-sulfur clusters, iron storage, iron absorption, mitochondrial iron homeostasis, mitochondrial disease
- MeSH terms
- Frataxin, Animals, Humans, Iron, Mitochondria, Ion Transport, ATP-Binding Cassette Transporters, Mitochondrial Diseases, Iron-Binding Proteins, Metabolic Networks and Pathways
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