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Antioxidants 2014, 3(3), 592-603;

Friedreich’s Ataxia: A Neuronal Point of View on the Oxidative Stress Hypothesis

Unit of Neuromuscular and Neurodegenerative diseases, Children's Hospital and Research Institute "Bambino Gesù", 00165 Rome, Italy
Authors to whom correspondence should be addressed.
Received: 23 March 2014 / Revised: 6 May 2014 / Accepted: 16 May 2014 / Published: 10 September 2014
(This article belongs to the Special Issue Oxidative Stress and Neurodegenerative Diseases)
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A prominent feature of Friedreich’s ataxia (FRDA) is the neurodegeneration of the central and peripheral nervous systems, but little information is available about the mechanisms leading to neuronal damage in this pathology. Currently, no treatments delay, prevent, or reverse the inexorable decline that occurs in this condition. Evidence of oxidative damage has been demonstrated in Friedreich’s ataxia, and this damage has been proposed as the origin of the disease. Nevertheless, the role of oxidative stress in FRDA remains debatable. The lack of direct evidence of reactive oxygen species overproduction in FRDA cells and tissues and the failure of exogenous antioxidants to rescue FRDA phenotypes questions the role of oxidative stress in this pathology. For example, the antioxidant “idebenone” ameliorates cardiomyopathy in FRDA patients, but this therapy does not improve neurodegeneration. To date, no known pharmacological treatment with antioxidant properties cures or delays FRDA neuropathology. This review reports and discusses the evidence of oxidative stress in FRDA and focuses on the existing knowledge of the apparent ineffectiveness of antioxidants for the treatment of neuronal damage. View Full-Text
Keywords: Friedreich’s ataxia; oxidative stress; neurodegeneration; antioxidants Friedreich’s ataxia; oxidative stress; neurodegeneration; antioxidants
This is an open access article distributed under the Creative Commons Attribution License (CC BY 3.0).

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Carletti, B.; Piemonte, F. Friedreich’s Ataxia: A Neuronal Point of View on the Oxidative Stress Hypothesis. Antioxidants 2014, 3, 592-603.

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