| Iron regulatory proteins secure mitochondrial iron sufficiency and function. | |
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MedLine Citation:
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PMID: 20674864 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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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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Authors:
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Bruno Galy; Dunja Ferring-Appel; Sven W Sauer; Sylvia Kaden; Saïd Lyoumi; Herve Puy; Stefan Kölker; Hermann-Josef Gröne; Matthias W Hentze |
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Publication Detail:
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Type: Journal Article; Research Support, Non-U.S. Gov't |
Journal Detail:
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Title: Cell metabolism Volume: 12 ISSN: 1932-7420 ISO Abbreviation: Cell Metab. Publication Date: 2010 Aug |
Date Detail:
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Created Date: 2010-08-02 Completed Date: 2010-11-22 Revised Date: - |
Medline Journal Info:
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Nlm Unique ID: 101233170 Medline TA: Cell Metab Country: United States |
Other Details:
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Languages: eng Pagination: 194-201 Citation Subset: IM |
Copyright Information:
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Copyright 2010 Elsevier Inc. All rights reserved. |
Affiliation:
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European Molecular Biology Laboratory, Meyerhofstrasse 1, 69117 Heidelberg, Germany. |
Export Citation:
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| MeSH Terms | |
Descriptor/Qualifier:
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Animals Energy Metabolism Heme / biosynthesis Iron / metabolism* Iron Regulatory Protein 1 / deficiency, genetics, metabolism Iron Regulatory Protein 2 / deficiency, genetics, metabolism Iron-Regulatory Proteins / deficiency, genetics, metabolism* Liver Failure / etiology, metabolism, pathology Mice Mice, Transgenic Mitochondria / metabolism* |
| Chemical | |
Reg. No./Substance:
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0/Iron-Regulatory Proteins; 14875-96-8/Heme; 7439-89-6/Iron; EC 4.2.1.3/Iron Regulatory Protein 1; EC 4.2.1.3/Iron Regulatory Protein 2 |
From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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