Regulation of Mitochondrial Iron Accumulation by Yfh1p, a Putative Homolog of Frataxin
Abstract
The gene responsible for Friedreich's ataxia, a disease characterized by neurodegeneration and cardiomyopathy, has recently been cloned and its product designated frataxin. A gene in Saccharomyces cerevisiae was characterized whose predicted protein product has high sequence similarity to the human frataxin protein. The yeast gene (yeast frataxin homolog, YFH1 ) encodes a mitochondrial protein involved in iron homeostasis and respiratory function. Human frataxin also was shown to be a mitochondrial protein. Characterizing the mechanism by which YFH1 regulates iron homeostasis in yeast may help to define the pathologic process leading to cell damage in Friedreich's ataxia.
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Disruption of yeast frataxin homologue YFH1 in yeast strain Δyfh1 induced accumulation of iron in mitochondria and enhanced sensitivity to oxidative stress by H2O2 and iron overload.
“For instance, the disruption of the yeast frataxin homologue (YFH1) in yeast strain (Δyfh1) induced an accumulation of iron in mitochondria and enhanced their sensitivity to oxidative stress (by H2O2 and iron overload) (13, 93).”
From Intracellular iron transport and storage: from molecular mechanisms to health implications (2008) · cites this paper as [13]
Deletion of yeast frataxin homologue YFH1 caused mitochondrial iron accumulation and increased sensitivity to H2O2 toxicity.
“The first evidence of frataxin's role in mitochondrial iron regulation was obtained by deletion of yeast frataxin homologue, YFH1, and characterization of the yeast phenotype, which revealed that the deletion of YFH1 caused mitochondrial iron accumulation and increased sensitivity to H2O2 toxicity (13).”
From Intracellular iron transport and storage: from molecular mechanisms to health implications (2008) · cites this paper as [13]
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- Authors
- Babcock, M., de Silva, D., Oaks, R., Davis-Kaplan, S., Jiralerspong, S., Montermini, L., Pandolfo, M., & Kaplan, J.
- Affiliations
- University of Utah (US)
- M. Babcock, D. de Silva, R. Oaks, S. Davis-Kaplan, J. Kaplan, Division of Immunology and Cell Biology, Department of Pathology, School of Medicine, University of Utah, Salt Lake City, UT 84132, USA.
- Corresponding author
- Kaplan, Jerry
- ORCID
- Pandolfo, Massimo
- Venue
- Science
- Published
- 1997-06-13
- Volume
- 276
- Issue
- 5319
- Pages
- 1709-1712
- ISSN
- 0036-8075 (ISSN-L)
- 1095-9203
- OpenAlex
- W2069849662
- Cited by
- 921
- References
- 25
- Citations by year
- 2026: 6 · 2025: 12 · 2024: 13 · 2023: 13 · 2022: 25 · 2021: 20 · 2020: 23 · 2019: 16 · 2018: 18 · 2017: 21 · 2016: 22 · 2015: 17 · 2014: 28 · 2013: 38 · 2012: 41
- FWCI
- 18.84
- Type
- journal article
- Language
- en
- Open access
- closed
- Locations
- Science (publishedVersion)
- PubMed (publishedVersion)
- Dépôt institutionnel de l'Université libre de Bruxelles (Université Libre de Bruxelles) (submittedVersion)
- Funders
- National Institutes of Health (5p50dk049219-05, 7r01ns034192-06)
- National Institute of Diabetes and Digestive and Kidney Diseases (DK-49219, DK30534)
- National Institute of Neurological Disorders and Stroke (NS34192)
- Topics
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Molecular Biology › Mitochondrial Function and Pathology
- Life Sciences › Neuroscience › Neurology › Neurological diseases and metabolism
- Life Sciences › Neuroscience › Cellular and Molecular Neuroscience › Genetic Neurodegenerative Diseases
- Keywords
- frataxin, Friedreich's ataxia, mitochondrial iron accumulation, iron homeostasis, Saccharomyces cerevisiae, mitochondrial proteins, respiratory function, neurodegeneration, cardiomyopathy
- MeSH terms
- Frataxin, Biological Transport, Carrier Proteins, Cell Membrane, Ceruloplasmin, Cytosol, Friedreich Ataxia, Fungal Proteins, Genes, Fungal, Genetic Complementation Test, Homeostasis, Humans, Iron, Mitochondria, Oxidoreductases, Recombinant Fusion Proteins, Saccharomyces cerevisiae, Transformation, Genetic, Phosphotransferases (Alcohol Group Acceptor), Oxidative Stress, Membrane Transport Proteins, Saccharomyces cerevisiae Proteins, 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.
Statistics and arithmetic are recomputed, not judged.
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