Skip to main content
Log in

Eisen-Schwefel-Protein-Biogenese: Was Mitochondrien essenziell macht

Schwerpunkt Mitochondrien

  • Wissenschaft
  • Published:
BIOspektrum Aims and scope

Abstract

Mitochondria are essential for the biosynthesis of Fe/S clusters (ISC) which serve as protein cofactors in enzyme catalysis, electron transfer and sensing. The mitochondrial ISC assembly machinery consists of 17 proteins which assist maturation of both organellar and cytosolic-nuclear Fe/S proteins. Some of the latter Fe/S proteins are indispensable for protein translation and nuclear DNA synthesis and repair. Thus, mitochondria perform a critical role in central processes of gene expression.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Similar content being viewed by others

Literatur

  1. Beinert H, Holm RH, Münck E (1997) Iron-sulfur clusters: Nature’s modular, multipurpose structures. Science 277:653–659

    Article  PubMed  CAS  Google Scholar 

  2. Kispal G, Sipos K, Lange H et al. (2005) Biogenesis of cytosolic ribosomes requires the essential iron-sulphur protein Rli1p and mitochondria. EMBO J 24:589–598

    Article  PubMed  CAS  Google Scholar 

  3. Becker T, Franckenberg S, Wickles S et al. (2012) Structural basis of highly conserved ribosome recycling in eukaryotes and archaea. Nature 482:501–506

    Article  PubMed  CAS  Google Scholar 

  4. Netz DJ, Stith CM, Stumpfig M et al. (2012) Eukaryotic DNA polymerases require an iron-sulfur cluster for the formation of active complexes. Nat Chem Biol 8:125–132

    Article  CAS  Google Scholar 

  5. Gari K, Leon Ortiz AM, Borel V et al. (2012) MMS19 links cytoplasmic iron-sulfur cluster assembly to DNA metabolism. Science 337:243–245

    Article  PubMed  CAS  Google Scholar 

  6. Stehling O, Vashisht AA, Mascarenhas J et al. (2012) MMS19 assembles iron-sulfur proteins required for DNA metabolism and genomic integrity. Science 337:195–199

    Article  PubMed  CAS  Google Scholar 

  7. Lill R, Hoffmann B, Molik S et al. (2012) The role of mitochondria in cellular iron-sulfur protein biogenesis and iron metabolism. Biochim Biophys Acta 1823:1491–1508

    Article  PubMed  CAS  Google Scholar 

  8. Lill R (2009) Function and biogenesis of iron-sulphur proteins. Nature 460:831–838

    Article  PubMed  CAS  Google Scholar 

  9. Sheftel A, Stehling O, Lill R (2010) Iron-sulfur proteins in health and disease. Trends Endocrinol Metab 21:302–314

    Article  PubMed  CAS  Google Scholar 

  10. Schmucker S, Puccio H (2010) Understanding the molecular mechanisms of Friedreich’s ataxia to develop therapeutic approaches. Hum Mol Genet 19:R103–110

    Article  PubMed  CAS  Google Scholar 

  11. Navarro-Sastre A, Tort F, Stehling O et al. (2011) A fatal mitochondrial disease is associated with defective NFU1 function in the maturation of a subset of mitochondrial Fe-S proteins. Am J Hum Genet 89:656–667

    Article  PubMed  CAS  Google Scholar 

  12. Cameron JM, Janer A, Levandovskiy V et al. (2011) Mutations in iron-sulfur cluster scaffold genes NFU1 and BOLA3 cause a fatal deficiency of multiple respiratory chain and 2-oxoacid dehydrogenase enzymes. Am J Hum Genet 89:486–495

    Article  PubMed  CAS  Google Scholar 

  13. Molik S, Lill R (2012) Role of mitosomes in cellular ironsulfur protein biogenesis. J Endocyt Cell Res 23:77–85

    Google Scholar 

  14. Goldberg AV, Molik S, Tsaousis AD et al. (2008) Localization and functionality of microsporidian iron-sulphur cluster assembly proteins. Nature 452:624–628

    Article  PubMed  CAS  Google Scholar 

Download references

Author information

Authors and Affiliations

Authors

Corresponding author

Correspondence to Roland Lill.

Additional information

Roland Lill Chemiestudium an den Universitäten Ulm und München. 1986 Promotion an der LMU München. 1987–1989 Postdoktorand als DFG-Stipendiat, University of California, Los Angeles, USA. 1990–1996 Gruppenleiter, Institut für Physiologische Chemie der LMU München. 1996 Habilitation. Seit 1996 Professor für Zellbiologie am Fachbereich Medizin der Universität Marburg.

Rights and permissions

Reprints and permissions

About this article

Cite this article

Lill, R. Eisen-Schwefel-Protein-Biogenese: Was Mitochondrien essenziell macht. Biospektrum 19, 128–130 (2013). https://doi.org/10.1007/s12268-013-0279-z

Download citation

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s12268-013-0279-z

Navigation