Send to

Choose Destination
Cell Metab. 2010 Aug 4;12(2):194-201. doi: 10.1016/j.cmet.2010.06.007.

Iron regulatory proteins secure mitochondrial iron sufficiency and function.

Author information

European Molecular Biology Laboratory, Meyerhofstrasse 1, 69117 Heidelberg, Germany.


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.

[Indexed for MEDLINE]
Free full text

Supplemental Content

Full text links

Icon for Elsevier Science
Loading ...
Support Center