Cited by 103Iron Metabolism and DisordersMitochondrial Function and PathologyNeurological diseases and metabolism

Mitochondrial Mayhem: The Mitochondrion as a Modulator of Iron Metabolism and Its Role in Disease

Huang, Michael Li-Hsuan, Lane, Darius J.R., Richardson, Des R.·2011·Antioxidants & Redox Signaling

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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Crossref · 10.1089/ars.2011.3921

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157 of 157 references verified0 citation errors · 0 not checkable

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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
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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