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.

Condo', I., Ventura, N., Malisan, F., Tomassini, B., Testi, R. (2006). A pool of extramitochondrial frataxin that promotes cell survival. THE JOURNAL OF BIOLOGICAL CHEMISTRY, 281(24), 16750-16756 [10.1074/jbc.M511960200].

A pool of extramitochondrial frataxin that promotes cell survival

CONDO', IVANO;MALISAN, FLORENCE;TESTI, ROBERTO
2006-06-16

Abstract

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.
16-giu-2006
Pubblicato
Rilevanza internazionale
Articolo
Sì, ma tipo non specificato
Settore MED/04 - PATOLOGIA GENERALE
English
Con Impact Factor ISI
Reactive Oxygen Species; Cell Line; Hela cells; oxidative stress; antioxidants; friedreich ataxia; humans; mitochondria; cell line, tumor; cell survival; apoptosis; iron-binding proteins; cytochromes c; jurkat Cells
Condo', I., Ventura, N., Malisan, F., Tomassini, B., Testi, R. (2006). A pool of extramitochondrial frataxin that promotes cell survival. THE JOURNAL OF BIOLOGICAL CHEMISTRY, 281(24), 16750-16756 [10.1074/jbc.M511960200].
Condo', I; Ventura, N; Malisan, F; Tomassini, B; Testi, R
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Utilizza questo identificativo per citare o creare un link a questo documento: https://hdl.handle.net/2108/8806
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