Monday, September 14, 2009

Friedreich's ataxia: Oxidative stress and cytoskeletal abnormalities

Journal of the Neurological Sciences
Article in Press, Corrected Proof


  Marco Sparacoa, 1, Laura Maria Gaetab, 1, Filippo Maria Santorellib, Chiara Passarellib, Giulia Tozzib, Enrico Bertinib, Alessandro Simonatic, Francesco Scaravillid, Franco Taronie, Charles Duyckaertsf, Michele Feleppaa and Fiorella Piemonteb, Corresponding Author Contact Information, E-mail The Corresponding Author


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.