Article
- The EMBO Journal (2006) 25, 174 - 183
- doi:10.1038/sj.emboj.7600905
Published online: 8 December 2005
Subject Category:
The Nfs1 interacting protein Isd11 has an essential role in Fe/S cluster biogenesis in mitochondria
Alexander C Adam1,a, Carsten Bornhövd1, Holger Prokisch2, Walter Neupert1 and Kai Hell1
- Institut für Physiologische Chemie, Universität München, München, Germany
- Institute of Human Genetics, Technical University Munich, GSF National Research Center for Environment and Health, Neuherberg, Germany
Correspondence to:
Kai Hell, Institut für Physiologische Chemie, Ludwig-Maximilians-Universität München, Butenandtstrasse 5, 81377 München, Germany. Tel.: +49 89 2180 77100; Fax: +49 89 2180 77093; E-mail: hell@med.uni-muenchen.de
aPresent address: Institut für Pathologie, Universität Bonn, Sigmund-Freud-Strasse 5, 53127 Bonn, Germany
Received 9 August 2005; Accepted 15 November 2005
Abstract
Formation of iron/sulfur (Fe/S) clusters, protein translocation and protein folding are essential processes in the mitochondria of Saccharomyces cerevisiae. In a systematic approach to characterize essential proteins involved in these processes, we identified a novel essential protein of the mitochondrial matrix, which is highly conserved from yeast to human and which we termed Isd11. Depletion of Isd11 caused a strong reduction in the levels of the Fe/S proteins aconitase and the Rieske protein, and a massive decrease in the enzymatic activities of aconitase and succinate dehydrogenase. Incorporation of iron into the Fe/S protein Leu1 and formation of the Fe/S cluster containing holoform of the mitochondrial ferredoxin Yah1 were inhibited in the absence of Isd11. This strongly suggests that Isd11 is required for the assembly of Fe/S proteins. We show that Isd11 forms a stable complex with Nfs1, the cysteine desulfurase of the mitochondrial machinery for Fe/S cluster assembly. In the absence of Isd11, Nfs1 is prone to aggregation. We propose that Isd11 acts together with Nfs1 in an early step in the biogenesis of Fe/S proteins.
Keywords:
- cysteine desulfurase,
- Fe/S cluster formation,
- Fe/S protein,
- Isd11,
- mitochondria
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