Jump to ContentJump to Main Navigation
Show Summary Details

Biological Chemistry

Editor-in-Chief: Brüne, Bernhard

Editorial Board Member: Buchner, Johannes / Lei, Ming / Ludwig, Stephan / Sies, Helmut / Turk, Boris / Wittinghofer, Alfred

12 Issues per year

IMPACT FACTOR 2015: 2.710
Rank 142 out of 289 in category Biochemistry & Molecular Biology in the 2015 Thomson Reuters Journal Citation Report/Science Edition

SCImago Journal Rank (SJR) 2015: 1.607
Source Normalized Impact per Paper (SNIP) 2015: 0.751
Impact per Publication (IPP) 2015: 2.609

See all formats and pricing
Volume 380, Issue 10 (Oct 1999)


The Essential Role of Mitochondria in the Biogenesis of Cellular Iron-Sulfur Proteins

Roland Lill / Kerstin Diekert / Anita Kaut / Heike Lange / Winfried Pelzer / Corinna Prohl / Gyula Kispal
Published Online: 2005-06-01 | DOI: https://doi.org/10.1515/BC.1999.147


Iron-sulfur (Fe/S) proteins play an important role in electron transfer processes and in various enzymatic reactions. In eukaryotic cells, known Fe/S proteins are localised in mitochondria, the cytosol and the nucleus. The biogenesis of these proteins has only recently become the focus of investigations. Mitochondria are the major site of Fe/S cluster biosynthesis in the cell. The organelles contain an Fe/S cluster biosynthesis apparatus that resembles that of prokaryotic cells. This apparatus consists of some ten proteins including a cysteine desulfurase producing elemental sulfur for biogenesis, a ferredoxin involved in reduction, and two chaperones. The mitochondrial Fe/S cluster synthesis apparatus not only assembles mitochondrial Fe/S proteins, but also initiates formation of extra-mitochondrial Fe/S proteins. This involves the export of sulfur and possibly iron from mitochondria to the cytosol, a reaction performed by the ABC transporter Atm1p of the mitochondrial inner membrane. A possible substrate of Atm1p is an Fe/S cluster that may be stabilised for transport. Constituents of the cytosol involved in the incorporation of the Fe/S cluster into apoproteins have not been described yet. Many of the mitochondrial proteins involved in Fe/S cluster formation are essential, illustrating the central importance of Fe/S proteins for life. Defects in Fe/S protein biogenesis are associated with the abnormal accumulation of iron within mitochondria and are the cause of an iron storage disease.

About the article

Published Online: 2005-06-01

Published in Print: 1999-10-12

Citation Information: Biological Chemistry, ISSN (Print) 1431-6730, DOI: https://doi.org/10.1515/BC.1999.147. Export Citation

Citing Articles

Here you can find all Crossref-listed publications in which this article is cited. If you would like to receive automatic email messages as soon as this article is cited in other publications, simply activate the “Citation Alert” on the top of this page.

Jerry Kaplan, Diane McVey Ward, Robert J. Crisp, and Caroline C. Philpott
Biochimica et Biophysica Acta (BBA) - Molecular Cell Research, 2006, Volume 1763, Number 7, Page 646
Khadine A. Higgins, Hui Peng, Justin L. Luebke, Feng-Ming James Chang, and David P. Giedroc
Biochemistry, 2015, Volume 54, Number 14, Page 2385
Heike Lange, Thomas Lisowsky, Jana Gerber, Ulrich Mühlenhoff, Gyula Kispal, and Roland Lill
EMBO reports, 2001, Volume 2, Number 8, Page 715
Matias S. Attene-Ramos, Ruili Huang, Sam Michael, Kristine L. Witt, Ann Richard, Raymond R. Tice, Anton Simeonov, Christopher P. Austin, and Menghang Xia
Environmental Health Perspectives, 2014
Xiaoling Chen, Zhiqing Huang, Bo Zhou, Huan Wang, Gang Jia, Guangmang Liu, and Hua Zhao
Endocrine, 2014, Volume 47, Number 2, Page 372
O. S. Chen, S. Hemenway, and J. Kaplan
Proceedings of the National Academy of Sciences, 2002, Volume 99, Number 26, Page 16922
Y. Ichikawa, M. Bayeva, M. Ghanefar, V. Potini, L. Sun, R. K. Mutharasan, R. Wu, A. Khechaduri, T. Jairaj Naik, and H. Ardehali
Proceedings of the National Academy of Sciences, 2012, Volume 109, Number 11, Page 4152
Matias S. Attene-Ramos, Ruili Huang, Srilatha Sakamuru, Kristine L. Witt, Gyda C. Beeson, Louie Shou, Rick G. Schnellmann, Craig C. Beeson, Raymond R. Tice, Christopher P. Austin, and Menghang Xia
Chemical Research in Toxicology, 2013, Volume 26, Number 9, Page 1323
Wenjing Xu, Tomasa Barrientos, and Nancy C. Andrews
Cell Metabolism, 2013, Volume 17, Number 3, Page 319
Emanuele Marzetti, Judy C.Y. Hwang, Hazel A. Lees, Stephanie E. Wohlgemuth, Esther E. Dupont-Versteegden, Christy S. Carter, Roberto Bernabei, and Christiaan Leeuwenburgh
Biochimica et Biophysica Acta (BBA) - General Subjects, 2010, Volume 1800, Number 3, Page 235
J. Frazzon and D. R. Dean
Proceedings of the National Academy of Sciences, 2001, Volume 98, Number 26, Page 14751
Winfried Pelzer, Ulrich Mühlenhoff, Kerstin Diekert, Kerstin Siegmund, Gyula Kispal, and Roland Lill
FEBS Letters, 2000, Volume 476, Number 3, Page 134
Shizue Katoh, Katsuyoshi Murata, Yoshiki Kubota, Hiroyuki Kumeta, Kenji Ogura, Fuyuhiko Inagaki, Munehiko Asayama, and Etsuko Katoh
Protein Expression and Purification, 2005, Volume 43, Number 2, Page 149
Helmut Beinert
European Journal of Biochemistry, 2000, Volume 267, Number 18, Page 5657
Taichi Isobe, Eishi Baba, Shuji Arita, Masato Komoda, Shingo Tamura, Tsuyoshi Shirakawa, Hiroshi Ariyama, Shigeo Takaishi, Hitoshi Kusaba, Takashi Ueki, and Koichi Akashi
Experimental Cell Research, 2011, Volume 317, Number 18, Page 2582
Roland Lill and Gyula Kispal
Research in Microbiology, 2001, Volume 152, Number 3-4, Page 331
Robert B Wilson
Journal of the Neurological Sciences, 2003, Volume 207, Number 1-2, Page 103
Xi-Ping Huang, Peter J. O’Brien, and Douglas M. Templeton
Chemico-Biological Interactions, 2006, Volume 163, Number 1-2, Page 68
Alessia Formigari, Paola Irato, and Alessandro Santon
Comparative Biochemistry and Physiology Part C: Toxicology & Pharmacology, 2007, Volume 146, Number 4, Page 443
Mark D. Fleming
Seminars in Hematology, 2002, Volume 39, Number 4, Page 270
Massimo Pandolfo
Blood Cells, Molecules, and Diseases, 2002, Volume 29, Number 3, Page 536
Mark van der Giezen
BioScience, 2011, Volume 61, Number 8, Page 594
Ulrich Mühlenhoff and Roland Lill
Biochimica et Biophysica Acta (BBA) - Bioenergetics, 2000, Volume 1459, Number 2-3, Page 370
Helmut Beinert
Biochimica et Biophysica Acta (BBA) - Bioenergetics, 2002, Volume 1553, Number 1-2, Page 7
Xiang Ming Xu and Simon Geir Møller
Antioxidants & Redox Signaling, 2011, Volume 15, Number 1, Page 271
Frank Seeber
International Journal for Parasitology, 2002, Volume 32, Number 10, Page 1207
Tobias Karlberg, Ulrika Schagerlöf, Oleksandr Gakh, Sungjo Park, Ulf Ryde, Martin Lindahl, Kirstin Leath, Elspeth Garman, Grazia Isaya, and Salam Al-Karadaghi
Structure, 2006, Volume 14, Number 10, Page 1535
Roland Lill and Gyula Kispal
Trends in Biochemical Sciences, 2000, Volume 25, Number 8, Page 352
Immo E. Scheffler
Advanced Drug Delivery Reviews, 2001, Volume 49, Number 1-2, Page 3
T Martin Embley
Philosophical Transactions of the Royal Society B: Biological Sciences, 2006, Volume 361, Number 1470, Page 1055
Biochimie, 2000, Volume 82, Number 6-7, Page 603
Chung-Yu Lan, Gabriel Rodarte, Luis A. Murillo, Ted Jones, Ronald W. Davis, Jan Dungan, George Newport, and Nina Agabian
Molecular Microbiology, 2004, Volume 53, Number 5, Page 1451
Fabien Pierrel, Paul A. Cobine, and Dennis R. Winge
BioMetals, 2007, Volume 20, Number 3-4, Page 675
J. M. Cooper and A. H. V. Schapira
BioFactors, 2003, Volume 18, Number 1-4, Page 163
M. Fontecave, S. Ollagnier de Choudens, B. Py, and F. Barras
JBIC Journal of Biological Inorganic Chemistry, 2005, Volume 10, Number 7, Page 713
M. Koc, Z. Nad'ová, and J. Kovář
Cell Proliferation, 2006, Volume 39, Number 6, Page 551
Matthias Schauen, Dimitry Spitkovsky, Jens Schubert, Jürgen H. Fischer, Jun-Ichi Hayashi, and Rudolf J. Wiesner
Journal of Cellular Physiology, 2006, Volume 209, Number 1, Page 103
Marcus E. Boyer, Chia-Wei Wang, and James R. Swartz
Biotechnology and Bioengineering, 2006, Volume 94, Number 1, Page 128
M. Koc, Z. Nad’ová, J. Truksa, M. Ehrlichová, and J. Kovář
Apoptosis, 2005, Volume 10, Number 2, Page 381
Gyula Kispal, Katalin Sipos, Heike Lange, Zsuzsanna Fekete, Tibor Bedekovics, Tamás Janáky, Jochen Bassler, Daili J Aguilar Netz, Janneke Balk, Carmen Rotte, and Roland Lill
The EMBO Journal, 2005, Volume 24, Number 3, Page 589
J. Truksa, J. Kovar, T. Valenta, M. Ehrlichova, J. Polak, and P. W. Naumann
Cell Proliferation, 2003, Volume 36, Number 4, Page 199
Massimo Pandolfo
Movement Disorders, 2001, Volume 16, Number 5, Page 815
Stuart A. Ralph, Giel G. van Dooren, Ross F. Waller, Michael J. Crawford, Martin J. Fraunholz, Bernardo J. Foth, Christopher J. Tonkin, David S. Roos, and Geoffrey I. McFadden
Nature Reviews Microbiology, 2004, Volume 2, Number 3, Page 203

Comments (0)

Please log in or register to comment.
Log in