Cited by 147Iron Metabolism and DisordersMitochondrial Function and PathologyNeurological diseases and metabolism

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Iron Regulatory Proteins Secure Mitochondrial Iron Sufficiency and Function

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Galy, Bruno, Ferring-Appel, Dunja, Sauer, Sven W., Kaden, Sylvia, Lyoumi, Saïd, Puy, Herve, Kölker, Stefan, Gröne, Hermann-Josef, Hentze, Matthias W.·2010·Cell Metabolism

DOI 10.1016/j.cmet.2010.06.007

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Abstract

Mitochondria supply cells with ATP, heme, and iron sulfur clusters (ISC), and mitochondrial energy metabolism involves both heme- and ISC-dependent enzymes. Here, we show that mitochondrial iron supply and function require iron regulatory proteins (IRP), cytosolic RNA-binding proteins that control mRNA translation and stability. Mice lacking both IRP1 and IRP2 in their hepatocytes suffer from mitochondrial iron deficiency and dysfunction associated with alterations of the ISC and heme biosynthetic pathways, leading to liver failure and death. These results uncover a major role of the IRPs in cell biology: to ensure adequate iron supply to the mitochondrion for proper function of this critical organelle.

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Additional file 2 of Diurnal control of iron responsive element containing mRNAs through iron regulatory proteins IRP1 and IRP2 is mediated by feeding rhythms
Nadimpalli, H. P., Katsioudi, G., Arpa, E. S., Chikhaoui, L., Arpat, A. B., Liechti, A., Palais, G., Tessmer, C., Hofmann, I., Galy, B., et al. (2024) · Figshare · doi:10.6084/m9.figshare.25876315
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Additional file 4 of Diurnal control of iron responsive element containing mRNAs through iron regulatory proteins IRP1 and IRP2 is mediated by feeding rhythms
Nadimpalli, H. P., Katsioudi, G., Arpa, E. S., Chikhaoui, L., Arpat, A. B., Liechti, A., Palais, G., Tessmer, C., Hofmann, I., Galy, B., et al. (2024) · Figshare · doi:10.6084/m9.figshare.26723735
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Additional file 1 of Diurnal control of iron responsive element containing mRNAs through iron regulatory proteins IRP1 and IRP2 is mediated by feeding rhythms
Nadimpalli, H. P., Katsioudi, G., Arpa, E. S., Chikhaoui, L., Arpat, A. B., Liechti, A., Palais, G., Tessmer, C., Hofmann, I., Galy, B., et al. (2024) · Figshare · doi:10.6084/m9.figshare.25876187
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Additional file 3 of Diurnal control of iron responsive element containing mRNAs through iron regulatory proteins IRP1 and IRP2 is mediated by feeding rhythms
Nadimpalli, H. P., Katsioudi, G., Arpa, E. S., Chikhaoui, L., Arpat, A. B., Liechti, A., Palais, G., Tessmer, C., Hofmann, I., Galy, B., et al. (2024) · Figshare · doi:10.6084/m9.figshare.26723732
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Authors: Galy, B., Ferring-Appel, D., Sauer, S. W., Kaden, S., Lyoumi, S., Puy, H., Kölker, S., Gröne, H.-J., & Hentze, M. W.
Affiliations: European Molecular Biology Laboratory (DE)
Heidelberg University (DE)
German Cancer Research Center (DE)
Corresponding author: Hentze, Matthias W.
ORCID: Galy, Bruno
Kölker, Stefan
Hentze, Matthias W.
Venue: Cell Metabolism
Publisher: Elsevier BV
Published: 2010-08
Volume: 12
Issue: 2
Pages: 194-201
ISSN: 1550-4131 (ISSN-L)
DOI: 10.1016/j.cmet.2010.06.007
OpenAlex: W1995039474
Cited by: 147
References: 39
Citations by year: 2026: 5 · 2025: 16 · 2024: 7 · 2023: 7 · 2022: 11 · 2021: 9 · 2020: 12 · 2019: 10 · 2018: 9 · 2017: 7 · 2016: 8 · 2015: 8 · 2014: 12 · 2013: 5 · 2012: 10
FWCI: 5.22
Type: journal article
Language: en
Open access: bronze
doi.org
Licence: www.elsevier.com/tdm/userlicense/1.0/ (tdm)
www.elsevier.com/open-access/userlicense/1.0/ (vor, 1081 d)
Locations: Cell Metabolism (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: iron regulatory proteins, iron regulatory protein 1, IRP2, iron deficiency, iron-sulfur clusters, heme biosynthesis, mitochondrial dysfunction, hepatocytes, liver failure, RNA-binding proteins
MeSH terms: Animals, Energy Metabolism, Heme, Iron, Mice, Transgenic, Mitochondria, Liver Failure, Iron-Regulatory Proteins, Iron Regulatory Protein 1, Iron Regulatory Protein 2, Mice

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Iron Regulatory Proteins Secure Mitochondrial Iron Sufficiency and Function

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