Article
- The EMBO Journal (2005) 24, 589 - 598
- doi:10.1038/sj.emboj.7600541
Published online: 20 January 2005
Subject Categories:
Biogenesis of cytosolic ribosomes requires the essential iron–sulphur protein Rli1p and mitochondria
Gyula Kispal1,2,a, Katalin Sipos1, Heike Lange2, Zsuzsanna Fekete1, Tibor Bedekovics1, Tamás Janáky3, Jochen Bassler4, Daili J Aguilar Netz2, Janneke Balk2, Carmen Rotte2 and Roland Lill2
- Institute of Biochemistry, University Medical School of Pecs, Hungary
- Institut für Zytobiologie und Zytopathologie der Philipps-Universität Marburg, Marburg, Germany
- Department of Medical Chemistry, Faculty of Medicine, University of Szeged, Szeged, Hungary
- Biochemie-Zentrum Heidelberg, Heidelberg, Germany
Correspondence to:
Roland Lill, Institut für Zytobiologie und Zytopathologie der Philipps-Universität Marburg, Robert-Koch-Str. 6, 35033 Marburg, Germany. Tel.: +49 6421 286 6449; Fax: +49 6421 286 6414; E-mail: Lill@mailer.uni-marburg.de
aDr Gyula Kispal died in a tragic car accident on March 20, 2003. We all miss him.
Received 29 April 2004; Accepted 8 December 2004
Abstract
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.
Keywords:
- ABC transporter,
- iron homeostasis,
- iron–sulphur cluster,
- nuclear export,
- protein translation
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