Skip Navigation



Human Molecular Genetics Advance Access published online on June 16, 2005
Human Molecular Genetics, doi:10.1093/hmg/ddi214
This Article
Right arrow FREE Full Text (PDF) Freely available
Right arrow All Versions of this Article:
14/15/2091    most recent
ddi214v1
Right arrow Alert me when this article is cited
Right arrow Alert me if a correction is posted
Services
Right arrow Email this article to a friend
Right arrow Similar articles in this journal
Right arrow Similar articles in PubMed
Right arrow Alert me to new issues of the journal
Right arrow Add to My Personal Archive
Right arrow Download to citation manager
Right arrowRequest Permissions
Google Scholar
Right arrow Articles by González-Cabo, P.
Right arrow Articles by Palau, F.
Right arrow Search for Related Content
PubMed
Right arrow PubMed Citation
Right arrow Articles by González-Cabo, P.
Right arrow Articles by Palau, F.
Social Bookmarking
Add to CiteULike   Add to Connotea   Add to Del.icio.us  
What's this?

© The Author 2005. Published by Oxford University Press. All rights reserved
Received March 16, 2005
Revised May 8, 2005
Accepted May 8, 2005

Article

Frataxin interacts functionally with mitochondrial electron transport chain proteins

Pilar González-Cabo 1, Rafael P. Vázquez-Manrique 2, M. Adelaida García-Gimeno 3, Pascual Sanz 3, and Francesc Palau 1*

1 Department of Genomics and Proteomics, Instituto de Biomedicina, CSIC, Valencia, Spain
2 Department of Genomics and Proteomics, Instituto de Biomedicina, CSIC, Valencia, Spain; Present address: Department of Zoology, University of Cambridge, Cambridge, UK
3 Department of Molecular and Cell Pathology and Therapy, Instituto de Biomedicina, CSIC, Valencia, Spain

* To whom correspondence should be addressed.
Francesc Palau, E-mail: fpalau{at}ibv.csic.es


  Abstract

Frataxin deficiency is the main cause of Friedreich ataxia, an autosomal recessive neurodegenerative disorder. Frataxin function in mitochondria has not been fully explained yet. In this work we show that Saccharomyces cerevisiae frataxin orthologue Yfh1p interacts physically with succinate dehydrogenase complex subunits Sdh1p and Sdh2p of the yeast mitochondrial electron transport chain, and also with ETF( and ETF{beta} subunits from the electron transfer flavoprotein complex. Genetic synthetic interaction experiments confirmed a functional relationship between YFH1 and succinate dehydrogenase genes SDH1 and SDH2. We also demonstrate a physical interaction between human frataxin and human succinate dehydrogenase complex subunits, suggesting also a key role of frataxin in the mitochondrial electron transport chain in humans. Consequently, we suggest a direct participation of the respiratory chain in the pathogenesis of the Friedreich ataxia, which we propose to be considered as an OXPHOS disease.


Add to CiteULike CiteULike   Add to Connotea Connotea   Add to Del.icio.us Del.icio.us    What's this?


This article has been cited by other articles:


Home page
 Proc. Natl. Acad. Sci. USAHome page
S. Long, M. Jirku, F. J. Ayala, and J. Lukes
Mitochondrial localization of human frataxin is necessary but processing is not for rescuing frataxin deficiency in Trypanosoma brucei
PNAS, September 9, 2008; 105(36): 13468 - 13473.
[Abstract] [Full Text] [PDF]

Home page
 Hum Mol GenetHome page
Y. Shan, E. Napoli, and G. Cortopassi
Mitochondrial frataxin interacts with ISD11 of the NFS1/ISCU complex and multiple mitochondrial chaperones
Hum. Mol. Genet., April 15, 2007; 16(8): 929 - 941.
[Abstract] [Full Text] [PDF]

Home page
 Proc. Natl. Acad. Sci. USAHome page
D. Pomplun, A. Voigt, T. J. Schulz, R. Thierbach, A. F. Pfeiffer, and M. Ristow
Reduced expression of mitochondrial frataxin in mice exacerbates diet-induced obesity
PNAS, April 10, 2007; 104(15): 6377 - 6381.
[Abstract] [Full Text] [PDF]

Home page
 J. Biol. Chem.Home page
H. Ding, J. Yang, L. C. Coleman, and S. Yeung
Distinct Iron Binding Property of Two Putative Iron Donors for the Iron-Sulfur Cluster Assembly: IscA AND THE BACTERIAL FRATAXIN ORTHOLOG CyaY UNDER PHYSIOLOGICAL AND OXIDATIVE STRESS CONDITIONS
J. Biol. Chem., March 16, 2007; 282(11): 7997 - 8004.
[Abstract] [Full Text] [PDF]

Home page
 FASEB J.Home page
J. V. Llorens, J. A. Navarro, M. J. Martinez-Sebastian, M. K. Baylies, S. Schneuwly, J. A. Botella, and M. D. Molto
Causative role of oxidative stress in a Drosophila model of Friedreich ataxia
FASEB J, February 1, 2007; 21(2): 333 - 344.
[Abstract] [Full Text] [PDF]

Home page
 J. Biol. Chem.Home page
Y. Zhang, E. R. Lyver, S. A. B. Knight, D. Pain, E. Lesuisse, and A. Dancis
Mrs3p, Mrs4p, and Frataxin Provide Iron for Fe-S Cluster Synthesis in Mitochondria
J. Biol. Chem., August 11, 2006; 281(32): 22493 - 22502.
[Abstract] [Full Text] [PDF]

Home page
 J. Biol. Chem.Home page
I. Condo, N. Ventura, F. Malisan, B. Tomassini, and R. Testi
A Pool of Extramitochondrial Frataxin That Promotes Cell Survival
J. Biol. Chem., June 16, 2006; 281(24): 16750 - 16756.
[Abstract] [Full Text] [PDF]

Home page
 J. Biol. Chem.Home page
V. Irazusta, E. Cabiscol, G. Reverter-Branchat, J. Ros, and J. Tamarit
Manganese Is the Link between Frataxin and Iron-Sulfur Deficiency in the Yeast Model of Friedreich Ataxia
J. Biol. Chem., May 5, 2006; 281(18): 12227 - 12232.
[Abstract] [Full Text] [PDF]

Home page
 J. Biol. Chem.Home page
T. J. Schulz, R. Thierbach, A. Voigt, G. Drewes, B. Mietzner, P. Steinberg, A. F. H. Pfeiffer, and M. Ristow
Induction of Oxidative Metabolism by Mitochondrial Frataxin Inhibits Cancer Growth: OTTO WARBURG REVISITED
J. Biol. Chem., January 13, 2006; 281(2): 977 - 981.
[Abstract] [Full Text] [PDF]

Home page
 Hum Mol GenetHome page
R. Thierbach, T. J. Schulz, F. Isken, A. Voigt, B. Mietzner, G. Drewes, J.-C. von Kleist-Retzow, R. J. Wiesner, M. A. Magnuson, H. Puccio, et al.
Targeted disruption of hepatic frataxin expression causes impaired mitochondrial function, decreased life span and tumor growth in mice
Hum. Mol. Genet., December 15, 2005; 14(24): 3857 - 3864.
[Abstract] [Full Text] [PDF]


Disclaimer: Please note that abstracts for content published before 1996 were created through digital scanning and may therefore not exactly replicate the text of the original print issues. All efforts have been made to ensure accuracy, but the Publisher will not be held responsible for any remaining inaccuracies. If you require any further clarification, please contact our Customer Services Department.