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Human Molecular Genetics Advance Access originally published online on June 16, 2005
Human Molecular Genetics 2005 14(15):2091-2098; doi:10.1093/hmg/ddi214
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© The Author 2005. Published by Oxford University Press. All rights reserved. For Permissions, please email: journals.permissions@oupjournals.org

Frataxin interacts functionally with mitochondrial electron transport chain proteins

Pilar González-Cabo1, Rafael P. Vázquez-Manrique1,{dagger}, M. Adelaida García-Gimeno2, Pascual Sanz2,{ddagger} and Francesc Palau1,{ddagger},*

1Department of Genomics and Proteomics and 2Department of Molecular and Cell Pathology and Therapy, Instituto de Biomedicina, CSIC, C/Jaume Roig 11, 46010 Valencia, Spain

* To whom correspondence should be addressed. Tel: +34 963393773; Fax: +34 963690800; Email: fpalau{at}ibv.csic.es

Received March 16, 2005; Accepted May 8, 2005

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 electron transfer flavoprotein complex ETF{alpha} and ETFß 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.

{dagger} Present address: Department of Zoology, University of Cambridge, Cambridge, UK.

{ddagger} These authors contributed equally to this work.


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