title: Friedreich's Ataxia: A Neuronal Point of View on the Oxidative Stress Hypothesis.
abstract: 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.
authors.affiliation: Unit of Neuromuscular and Neurodegenerative diseases, Children's Hospital and Research Institute "Bambino Gesù", 00165 Rome, Italy. carletti.barbara@tiscali.it.
authors.forename: Barbara
authors.initials: B
authors.lastname: Carletti
authors.affiliation: Unit of Neuromuscular and Neurodegenerative diseases, Children's Hospital and Research Institute "Bambino Gesù", 00165 Rome, Italy. fiorella.piemonte@opbg.net.
authors.forename: Fiorella
authors.initials: F
authors.lastname: Piemonte
keywords: Friedreich’s ataxia; antioxidants; neurodegeneration; oxidative stress