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Journal Articles BMC Neurology Year : 2008

Deferiprone targets aconitase: Implication for Friedreich's ataxia treatment.

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ABSTRACT: BACKGROUND: Friedreich ataxia is a neurological disease originating from an iron-sulfur cluster enzyme deficiency due to impaired iron handling in the mitochondrion, aconitase being particularly affected. As a mean to counteract disease progression, it has been suggested to chelate free mitochondrial iron. Recent years have witnessed a renewed interest in this strategy because of availability of deferiprone, a chelator preferentially targeting mitochondrial iron. METHOD: Control and Friedreich's ataxia patient cultured skin fibroblasts, frataxin-depleted neuroblastoma-derived cells (SK-N-AS) were studied for their response to iron chelation, with a particular attention paid to iron-sensitive aconitase activity. RESULTS: We found that a direct consequence of chelating mitochondrial free iron in various cell systems is a concentration and time dependent loss of aconitase activity. Impairing aconitase activity was shown to precede decreased cell proliferation. CONCLUSION: We conclude that, if chelating excessive mitochondrial iron may be beneficial at some stage of the disease, great attention should be paid to not fully deplete mitochondrial iron store in order to avoid undesirable consequences.
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inserm-00292152 , version 1 (30-06-2008)



Sergio Goncalves, Vincent Paupe, Emmanuel P. Dassa, Pierre Rustin. Deferiprone targets aconitase: Implication for Friedreich's ataxia treatment.. BMC Neurology, 2008, 8 (20), ⟨10.1186/1471-2377-8-20⟩. ⟨inserm-00292152⟩
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