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Human Molecular Genetics, 2001, Vol. 10, No. 19 2061-2067
© 2001 Oxford University Press

Disabled early recruitment of antioxidant defenses in Friedreich’s ataxia

Karine Chantrel-Groussard, Vanna Geromel, Hélène Puccio1, Michel Koenig1, Arnold Munnich, Agnès Rötig and Pierre Rustin+

Unité de Recherches sur les Handicaps Génétiques de l’Enfant, INSERM U393, Tour Lavoisier, Hôpital Necker-Enfants-Malades, 149 rue de Sèvres, 75743 Paris Cedex 15, France and 1Institut de Génétique et de Biologie Moléculaire et Cellulaire, INSERM, CNRS, 1 rue Laurent Fries, BP 163, 67404 Illkirch, France

Friedreich’s ataxia (FRDA) results from a generalized deficiency of mitochondrial iron–sulfur protein activity ascribed to mitochondrial iron overload. However, iron overload appears to be a late event in the disease. Here we show that neither superoxide dismutases nor the import iron machinery was induced by an endogenous oxidative stress in FRDA patients’ fibroblasts in contrast to control cells. Superoxide dismutase activity was not induced in the heart of conditional frataxin-KO mice either. This suggests that continuous oxidative damage to iron–ulfur clusters, resulting from hampered superoxide dismutase signaling, is causative of the mitochondrial deficiency and long term mitochondrial iron overload occurring in FRDA.

+ To whom correspondence should be addressed. Tel: +33 1 44 49 51 51; Fax: +33 1 47 34 85 14; Email: rustin@necker.fr


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