Protective effects of Fe-Aox29, a novel antioxidant derived from a molecular combination of Idebenone and vitamin E, in immortalized fibroblasts and fibroblasts from patients with Friedreich Ataxia.
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| Abstract |
Friedreich Ataxia (FRDA), the most frequent inherited ataxia, is not only characterized by progressive gait and limb ataxia, but in most cases is also accompanied by a severe hypertrophic cardiomyopathy. This life threatening symptom can be ameliorated by the administration of idebenone, a short chain quinone antioxidant, supporting additional evidence that oxidative stress plays a major role in the pathogenesis of this disease. In this study we analyze the combinatorial effect of different antioxidants on cell viability of FRDA fibroblasts and of RAT-1 immortalized fibroblasts exposed to oxidative stress. We find that an equimolar mixture of idebenone and vitamin E is more potent than each of the compound alone. Increased potency was also obtained with a novel synthetic antioxidant (Fe-Aox29) combining the active groups from both idebenone and vitamin E. These results indicate, that idebenone and vitamin E might act synergistically to counteract oxidative stress in fibroblasts from FRDA patients.
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| Authors | Matthias L Jauslin, Silvia Vertuani, Elisa Durini, Lisa Buzzoni, Nunzia Ciliberti, Sara Verdecchia, Paola Palozza, Thomas Meier, Stefano Manfredini |
| Journal | Molecular and cellular biochemistry (Mol Cell Biochem) Vol. 302 Issue 1-2 Pg. 79-85 (Aug 2007) ISSN: 0300-8177 [Print] Netherlands |
| PMID | 17476463 (Publication Type: Journal Article, Research Support, Non-U.S. Gov't) |
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