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Essential role of Isd11 in mitochondrial iron–sulfur cluster synthesis on Isu scaffold proteins
Author(s) -
Wiedemann Nils,
Urzica Eugen,
Guiard Bernard,
Müller Hanne,
Lohaus Christiane,
Meyer Helmut E,
Ryan Michael T,
Meisinger Chris,
Mühlenhoff Ulrich,
Lill Roland,
Pfanner Nikolaus
Publication year - 2006
Publication title -
the embo journal
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 7.484
H-Index - 392
eISSN - 1460-2075
pISSN - 0261-4189
DOI - 10.1038/sj.emboj.7600906
Subject(s) - humanities , library science , philosophy , art history , classics , art , computer science
Mitochondria are indispensable for cell viability; however, major mitochondrial functions including citric acid cycle and oxidative phosphorylation are dispensable. Most known essential mitochondrial proteins are involved in preprotein import and assembly, while the only known essential biosynthetic process performed by mitochondria is the biogenesis of iron–sulfur clusters (ISC). The components of the mitochondrial ISC‐assembly machinery are derived from the prokaryotic ISC‐assembly machinery. We have identified an essential mitochondrial matrix protein, Isd11 (YER048w‐a), that is found in eukaryotes only. Isd11 is required for biogenesis of cellular Fe/S proteins and thus is a novel subunit of the mitochondrial ISC‐assembly machinery. It forms a complex with the cysteine desulfurase Nfs1 and is required for formation of an Fe/S cluster on the Isu scaffold proteins. We conclude that Isd11 is an indispensable eukaryotic component of the mitochondrial machinery for biogenesis of Fe/S proteins.