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J. Biol. Chem., Vol. 281, Issue 24, 16750-16756, June 16, 2006

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A Pool of Extramitochondrial Frataxin That Promotes Cell Survival^*

Ivano Condò¹, Natascia Ventura¹, Florence Malisan, Barbara Tomassini, and Roberto Testi²

From the Laboratory of Signal Transduction, Department of Experimental Medicine and Biochemical Sciences, University of Rome "Tor Vergata," via Montpellier 1, 00133 Rome, Italy and the Fondazione Santa Lucia, via Ardeatina, 306-00179 Rome, Italy

Frataxin is a mitochondrial protein involved in iron metabolism. Defective expression of frataxin causes Friedreich ataxia (FA), an inherited degenerative syndrome characterized by ataxia, cardiomyopathy, and high incidence of diabetes. Here we report that frataxin-deficient cells are more prone to undergo stress-induced mitochondrial damage and apoptosis, while the overexpression of frataxin confers protection to a variety of cell types. Moreover, we reveal the existence of an extramitochondrial pool of frataxin, which can efficiently prevent mitochondrial damage and apoptosis in different cellular systems. Remarkably, extramitochondrial frataxin can fully replace mitochondrial frataxin in promoting survival of FA cells.

Received for publication, November 7, 2005 , and in revised form, March 31, 2006.

^* This work was supported by the Associazione Italiana Ricerca sul Cancro and by the European Commission, Projects "CELLAGE" and "TRANSDEATH." The costs of publication of this article were defrayed in part by the payment of page charges. This article must therefore be hereby marked "advertisement" in accordance with 18 U.S.C. Section 1734 solely to indicate this fact.

¹ These authors contributed equally to this work.

² To whom correspondence should be addressed. E-mail: roberto.testi{at}uniroma2.it.

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