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

Antioxidants
Barbara Carletti, Fiorella Piemonte

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.

References

Jan 1, 1985·Advances in Enzyme Regulation·E Cadenas, H Sies
Feb 7, 1998·Current Opinion in Neurobiology·M Koenig, J L Mandel
Feb 13, 1999·The Journal of Biological Chemistry·D C RadiskyJ Kaplan
Sep 29, 1999·Proceedings of the National Academy of Sciences of the United States of America·R LodiA H Schapira
Dec 23, 1999·Human Molecular Genetics·J L BradleyA H Schapira
Sep 15, 2000·Archives of Neurology·N Delanty, M A Dichter
Dec 13, 2000·Neurology·J B SchulzM B Bogdanov
Dec 13, 2000·Neurology·M EmondM Pandolfo
Sep 14, 2001·Human Molecular Genetics·M M SantosM Pandolfo
Oct 9, 2001·Human Molecular Genetics·K Chantrel-GroussardP Rustin
Dec 12, 2001·European Journal of Clinical Investigation·F PiemonteE Bertini
Feb 2, 2002·Human Molecular Genetics·Patrizia CavadiniGrazia Isaya
Apr 24, 2002·Archives of Disease in Childhood·G TozziF Piemonte
Jun 19, 2002·Free Radical Research·Pierre RustinDaniel Sidi
Oct 24, 2002·Human Molecular Genetics·Luigi PianeseSergio Cocozza
Aug 19, 2003·FASEB Journal : Official Publication of the Federation of American Societies for Experimental Biology·Matthias L JauslinMichael P Murphy
Mar 27, 2004·Biochemical and Biophysical Research Communications·Jean-Jacques BrièrePierre Rustin
May 5, 2004·The Journal of Biological Chemistry·Taejin Yoon, J A Cowan
Nov 19, 2005·FASEB Journal : Official Publication of the Federation of American Societies for Experimental Biology·Rafael P Vázquez-ManriqueFrancesc Palau
Apr 13, 2006·The Journal of Biological Chemistry·Ivano CondòRoberto Testi
Dec 15, 2006·FASEB Journal : Official Publication of the Federation of American Societies for Experimental Biology·José V LlorensMaria D Moltó
Jun 29, 2007·Human Molecular Genetics·Alain MartelliHélène Puccio
Jan 11, 2008·Proceedings of the National Academy of Sciences of the United States of America·Peter R AndersonJohn P Phillips
Jun 20, 2008·Human Molecular Genetics·Françoise AuchèreJean-Michel Camadro
Apr 11, 2009·Journal of Neurology·Massimo Pandolfo
Apr 11, 2009·Journal of Neurology·Massimo Pandolfo, Annalisa Pastore
Sep 4, 2009·Acta Neuropathologica·Arnulf H KoeppenDanhong Li
Sep 15, 2009·Journal of the Neurological Sciences·Marco SparacoFiorella Piemonte
Dec 10, 2009·Journal of Molecular Medicine : Official Organ of the Gesellschaft Deutscher Naturforscher Und Ärzte·Des R RichardsonYohan Suryo Rahmanto
Feb 17, 2010·Antioxidants & Redox Signaling·Renata SantosEmmanuel Lesuisse
Apr 13, 2010·PloS One·Daniele MarmolinoMassimo Pandolfo
Aug 27, 2010·The Journal of Neuroscience : the Official Journal of the Society for Neuroscience·Yujiro Shidara, Peter J Hollenbeck

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