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EMBO reports 4, 9, 906–911 (2003)
doi:10.1038/sj.embor.embor918
AOP Published online: 15 August 2003
An interaction between frataxin and Isu1/Nfs1 that is crucial for Fe/S cluster synthesis on Isu1
Jana Gerber, Ulrich Mühlenhoff & Roland Lill
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Institut für Zytobiologie und
Zytopathologie, Philipps-Universität Marburg, Robert-Koch
Strasse 6, D-35033 Marburg,
Germany
To whom correspondence should be addressed
Roland Lill Tel: +49 6421 286 6449; Fax: +49 6421 286 6414;
lill@mailer.uni-marburg.de
Received 19 May 2003; Accepted 14 July 2003; Published online 15 August 2003.
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Abstract
Depletion of the mitochondrial matrix protein frataxin is the
molecular cause of the neurodegenerative disease Friedreich ataxia. The
function of frataxin is unclear, although recent studies have suggested a
function of frataxin (yeast Yfh1) in iron/sulphur (Fe/S) protein biogenesis.
Here, we show that Yfh1 specifically binds to the central Fe/S-cluster
(ISC)-assembly complex, which is composed of the scaffold protein Isu1 and the
cysteine desulphurase Nfs1. Association between Yfh1 and Isu1/Nfs1 was markedly
increased by ferrous iron, but did not depend on ISCs on Isu1. Functional
analyses in vivo showed an involvement of Yfh1 in de novo ISC
synthesis on Isu1. Our data demonstrate a crucial function of Yfh1 in Fe/S
protein biogenesis by defining its function in an early step of this essential
process. The iron-dependent binding of Yfh1 to Isu1/Nfs1 suggests a role of
frataxin/Yfh1 in iron loading of the Isu scaffold proteins.
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