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Biological Chemistry

Role Of Iron Regulatory Proteins Ironed Out

On top of other functions, IRPs make it possible for cell mitochondria to maintain proper iron balance

by Stuart A. Borman
August 16, 2010 | A version of this story appeared in Volume 88, Issue 33

Iron regulatory proteins (IRPs) control cellular iron levels, and their absence or malfunction can be deadly, researchers report. The work could have implications in the treatment of Friedreich’s ataxia (a neurodegenerative condition) and a heritable form of anemia, both of which arise from disturbances of cellular iron balance. Proper iron balance in cells is essential because the element is needed to biosynthesize heme, iron-sulfur clusters in enzymes, and other structures, but the metal can be toxic in excess. IRPs were earlier found to regulate key iron-metabolism genes, but their overall function in cell physiology was unknown. Matthias W. Hentze of the European Molecular Biology Laboratory, in Heidelberg, Germany, and coworkers now report that IRPs make it possible for cell mitochondria to maintain proper iron balance (Cell Metab. 2010, 12, 194). They show that mice that don’t express the proteins in their liver cells develop mitochondrial iron deficiency, leading to liver failure and death. The IRP system “plays a critical role for mitochondrial iron management and function and is essential for liver physiology and organismal survival,” the researchers write.

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