Iron Regulatory Proteins Secure Mitochondrial Iron Sufficiency and Function

被引：105

作者：

Galy, Bruno ^{[1
]}

Ferring-Appel, Dunja ^{[1
]}

Sauer, Sven W. ^{[2
]}

Kaden, Sylvia ^{[3
]}

Lyoumi, Said ^{[4
,5
]}

Puy, Herve ^{[4
,5
]}

Koelker, Stefan ^{[2
]}

Groene, Hermann-Josef ^{[3
]}

Hentze, Matthias W. ^{[1
]}

机构：

[1] European Mol Biol Lab, D-69117 Heidelberg, Germany

[2] Univ Childrens Hosp Heidelberg, Div Inherited Metab, Dept Gen Pediat, D-69120 Heidelberg, Germany

[3] Deutsch Krebsforschungszentrum Heidelberg, D-69120 Heidelberg, Germany

[4] Hop Louis Mourier, Ctr Francais Porphyries, F-92701 Colombes, France

[5] Univ Versailles St Quentin Yvelines, Ctr Rech Biomed Bichat Beaujon, INSERM, U773, F-75018 Paris, France

来源：

CELL METABOLISM | 2010年 / 12卷 / 02期

关键词：

SULFUR CLUSTER BIOGENESIS; GENE-EXPRESSION; DEFICIENT MICE; HOMEOSTASIS; HEME; METABOLISM; LIVER; RAT; BIOSYNTHESIS; TRANSFERRIN;

D O I：

10.1016/j.cmet.2010.06.007

中图分类号：

Q2 [细胞生物学];

学科分类号：

071009 ; 090102 ;

摘要：

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.

引用

页码：194 / 201

页数：8

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