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

Gyula Kispal, Katalin Sipos, Heike Lange, Zsuzsanna Fekete, Tibor Bedekovics, Tamás Janáky, Jochen Bassler, Daili J. Aguilar Netz, Janneke Balk, Carmen Rotte, Roland Lill

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205 Citations (Scopus)


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.
Original languageEnglish
Pages (from-to)589-598
Number of pages10
JournalThe EMBO Journal
Issue number3
Publication statusPublished - Jan 2005

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