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Iron regulatory proteins secure mitochondrial iron sufficiency and function

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 m...

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Hauptverfasser: Galy, Bruno (VerfasserIn) , Ferring-Appel, Dunja (VerfasserIn) , Sauer, Sven (VerfasserIn) , Kaden, Sylvia (VerfasserIn) , Lyoumi, Saïd (VerfasserIn) , Puy, Herve (VerfasserIn) , Kölker, Stefan (VerfasserIn) , Gröne, Hermann-Josef (VerfasserIn) , Hentze, Matthias W. (VerfasserIn)
Dokumenttyp: Article (Journal)
Sprache:Englisch
Veröffentlicht: August 3, 2010
In: Cell metabolism
Year: 2010, Jahrgang: 12, Heft: 2, Pages: 194-201
ISSN:1932-7420
DOI:10.1016/j.cmet.2010.06.007
Online-Zugang:Verlag, lizenzpflichtig, Volltext: https://doi.org/10.1016/j.cmet.2010.06.007
Verlag, lizenzpflichtig, Volltext: https://www.sciencedirect.com/science/article/pii/S1550413110002305
Volltext
Verfasserangaben:Bruno Galy, Dunja Ferring-Appel, Sven W. Sauer, Sylvia Kaden, Saïd Lyoumi, Herve Puy, Stefan Kölker, Hermann-Josef Gröne, and Matthias W. Hentze
Beschreibung
Zusammenfassung: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.
Beschreibung:Gesehen am 25.07.2023
Beschreibung:Online Resource
ISSN:1932-7420
DOI:10.1016/j.cmet.2010.06.007

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