Cited by 152Metalloenzymes and iron-sulfur proteinsMitochondrial Function and PathologyNeurological diseases and metabolism

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Inhibition of Fe-S cluster biosynthesis decreases mitochondrial iron export: Evidence that Yfh1p affects Fe-S cluster synthesis

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Chen, Opal S., Hemenway, Shawn, Kaplan, Jerry·2002·Proceedings of the National Academy of Sciences

DOI 10.1073/pnas.192449599PMC PMC129443

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Abstract

Decreased expression of Yfh1p in the budding yeast, Saccharomyces cerevisiae , and the orthologous human gene frataxin results in respiratory deficiency and mitochondrial iron accumulation. The absence of Yfh1p decreases mitochondrial iron export. We demonstrate that decreased expression of Nfs1p, the yeast cysteine desulfurase that plays a central role in Fe-S cluster synthesis, also results in mitochondrial iron accumulation due to decreased export of mitochondrial iron. In the absence of Yfh1p, activity of Fe-S-containing enzymes (aconitase, succinate dehydrogenase) is decreased, whereas the activity of a non-Fe-S-containing enzyme (malate dehydrogenase) is unaffected. Aconitase protein was abundant even though the activity of aconitase was decreased in both aerobic and anaerobic conditions. These results demonstrate a direct role of Yfh1p in the formation of Fe-S clusters and indicate that mitochondrial iron export requires Fe-S cluster biosynthesis.

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Authors: Chen, O. S., Hemenway, S., & Kaplan, J.
Affiliations: University of Utah (US)
ORCID: Chen, Opal S.
Venue: Proceedings of the National Academy of Sciences
Publisher: National Academy of Sciences
Published: 2002-09-09
Volume: 99
Issue: 19
Pages: 12321-12326
ISSN: 0027-8424 (ISSN-L)
1091-6490
DOI: 10.1073/pnas.192449599
PMC: PMC129443
OpenAlex: W2039280518
Cited by: 152
References: 31
Citations by year: 2026: 1 · 2025: 1 · 2024: 3 · 2023: 3 · 2022: 1 · 2021: 8 · 2020: 5 · 2019: 4 · 2018: 2 · 2017: 5 · 2015: 2 · 2014: 5 · 2013: 8 · 2012: 7
FWCI: 16.64
Type: journal article
Language: en
Open access: green
www.ncbi.nlm.nih.gov
Locations: Proceedings of the National Academy of Sciences (publishedVersion)
PubMed Central (publishedVersion)
PubMed (publishedVersion)
Funders: National Institutes of Health
National Institute of Diabetes and Digestive and Kidney Diseases (DK 52380, R01DK052380)
Topics: Life Sciences › Biochemistry, Genetics and Molecular Biology › Biochemistry › Metalloenzymes and iron-sulfur proteins
Life Sciences › Biochemistry, Genetics and Molecular Biology › Molecular Biology › Mitochondrial Function and Pathology
Life Sciences › Neuroscience › Neurology › Neurological diseases and metabolism
Keywords: frataxin, iron-sulfur cluster assembly, mitochondrial iron accumulation, Saccharomyces cerevisiae, aconitase, succinate dehydrogenase, malate dehydrogenase, respiratory deficiency, cysteine desulfurase
MeSH terms: Frataxin, Aconitate Hydratase, Biological Transport, Active, Carrier Proteins, Fungal Proteins, Humans, Iron, Iron-Sulfur Proteins, Mitochondria, Oxidation-Reduction, RNA, Fungal, RNA, Messenger, Saccharomyces cerevisiae, Sulfurtransferases, Mitochondrial Proteins, Saccharomyces cerevisiae Proteins, Iron-Binding Proteins

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Inhibition of Fe-S cluster biosynthesis decreases mitochondrial iron export: Evidence that Yfh1p affects Fe-S cluster synthesis

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