Friedreich's ataxia: Oxidative stress and cytoskeletal abnormalities

Journal of the Neurological Sciences
Article in Press, Corrected Proof

  Marco Sparaco^{a, 1}, Laura Maria Gaeta^{b, 1}, Filippo Maria Santorelli^b, Chiara Passarelli^b, Giulia Tozzi^b, Enrico Bertini^b, Alessandro Simonati^c, Francesco Scaravilli^d, Franco Taroni^e, Charles Duyckaerts^f, Michele Feleppa^a and Fiorella Piemonte^{b, ,}

^aDivision of Neurology, Department of Neurosciences, Azienda Ospedaliera “G. Rummo”, 82100 Benevento, Italy
^bMolecular Medicine Unit, Children's Hospital and Research Institute “Bambino Gesù”, Roma, Italy
^cDepartment of Neurological and Visual Sciences, Section of Neurology, University of Verona, Policlinico G.B. Rossi, 37134 Verona, Italy
^dDivision of Neuropathology, Institute of Neurology, University College London, WC1N 3BG London, UK
^eUO Biochimica e Genetica, Fondazione IRCCS Istituto Neurologico “Carlo Besta”, Milano, Italy
^fLaboratoire de Neuropathologie Raymond Escourolle, Hôpital de La Salpêtrière, 75651 Paris, France

Received 2 April 2009; 

revised 24 July 2009; 

accepted 13 August 2009. 

Available online 12 September 2009.

Keywords: Glutathione; Oxidative stress; Friedreich's ataxia; Cytoskeletal proteins,  frataxin, Complexes I, II, III,  aconitase, fibroblasts, tubulin,  neurofilaments, motor neurons.