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.

Paola Palozza, Silvia Vertuani, Elisa Durini, Lisa Buzzoni, Nunzia Ciliberti, Sara Verdecchia, Thomas Meier, Matthias L. Jauslin, Stefano Manfredini

Risultato della ricerca: Contributo in rivistaArticolo in rivistapeer review

15 Citazioni (Scopus)

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.
Lingua originaleEnglish
pagine (da-a)79-85
Numero di pagine7
RivistaMolecular and Cellular Biochemistry
Stato di pubblicazionePubblicato - 2007
Pubblicato esternamenteYes

Keywords

  • Fe-Aox29
  • Fibroblasts
  • Friedreich Ataxia
  • Idebenone
  • Oxidative stress
  • Vitamin E

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