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 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.


Projects: B1.3: Linking modulation of iron metabolism with the impact of macrophag..., HepatoSys

Cell Metab.
Cell Metab. 12(2): 194-201
3rd Aug 2010

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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[Bruno Galy] [Matthias Hentze]

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Views: 1383
  • Created: 29th Oct 2012 at 11:00
  • Last updated: 24th Oct 2013 at 16:20

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