Biogenesis of cytosolic ribosomes requires the essential iron-sulphur protein Rli1p and mitochondria

被引:199
作者
Kispal, G
Sipos, K
Lange, H
Fekete, Z
Bedekovics, T
Janáky, T
Bassler, J
Netz, DJA
Balk, J
Rotte, C
Lill, R
机构
[1] Univ Marburg, Inst Zytobiol & Zytopathol, D-35033 Marburg, Germany
[2] Univ Med Sch Pecs, Inst Biochem, Pecs, Hungary
[3] Univ Szeged, Fac Med, Dept Med Chem, Szeged, Hungary
[4] Biochem Zentrum Heidelberg, Heidelberg, Germany
关键词
ABC transporter; iron homeostasis; iron-sulphur cluster; nuclear export; protein translation;
D O I
10.1038/sj.emboj.7600541
中图分类号
Q5 [生物化学]; Q7 [分子生物学];
学科分类号
071010 ; 081704 ;
摘要
Mitochondria perform a central function in the biogenesis of cellular iron - sulphur (Fe/S) proteins. It is unknown to date why this biosynthetic pathway is indispensable for life, the more so as no essential mitochondrial Fe/S proteins are known. Here, we show that the soluble ATP-binding cassette (ABC) protein Rli1p carries N-terminal Fe/S clusters that require the mitochondrial and cytosolic Fe/ S protein biogenesis machineries for assembly. Mutations in critical cysteine residues of Rli1p abolish association with Fe/ S clusters and lead to loss of cell viability. Hence, the essential character of Fe/ S clusters in Rli1p explains the indispensable character of mitochondria in eukaryotes. We further report that Rli1p is associated with ribosomes and with Hcr1p, a protein involved in rRNA processing and translation initiation. Depletion of Rli1p causes a nuclear export defect of the small and large ribosomal subunits and subsequently a translational arrest. Thus, ribosome biogenesis and function are intimately linked to the crucial role of mitochondria in the maturation of the essential Fe/ S protein Rli1p.
引用
收藏
页码:589 / 598
页数:10
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