Essential role of Isd11 in mitochondrial iron-sulfur cluster synthesis on Isu scaffold proteins

Nils Wiedemann, Eugen Urzica, Bernard Guiard, Hanne Müller, Christiane Lohaus, Helmut E. Meyer, Michael T. Ryan, Chris Meisinger, Ulrich Mühlenhoff, Roland Lill, Nikolaus Pfanner

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174 Citations (Scopus)


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.

Original languageEnglish
Pages (from-to)184-195
Number of pages12
JournalThe EMBO Journal
Issue number1
Publication statusPublished - 11 Jan 2006
Externally publishedYes


  • Essential cellular functions
  • Fe/S proteins
  • Mitochondria
  • Saccharomyces cerevisiae

Cite this

Wiedemann, N., Urzica, E., Guiard, B., Müller, H., Lohaus, C., Meyer, H. E., Ryan, M. T., Meisinger, C., Mühlenhoff, U., Lill, R., & Pfanner, N. (2006). Essential role of Isd11 in mitochondrial iron-sulfur cluster synthesis on Isu scaffold proteins. The EMBO Journal, 25(1), 184-195.