TAILIEUCHUNG - Báo cáo khoa học: Antioxidant defences in cybrids harboring mtDNA mutations associated with Leber’s hereditary optic neuropathy

Oxidative stress and imbalance between free radical generation and detoxi-fication may play a pivotal role in the pathogenesis of Leber’s hereditary optic neuropathy (LHON). Mitochondria, carrying the homoplasmic 11778⁄ND4, 3460⁄ND1 and 14484⁄ND6 mtDNA point mutations associ-ated with LHON, were used to generate osteosarcoma-derived cybrids. Enhanced mitochondrial production of reactive oxygen species has recently been demonstrated in these cybrids [Beretta S, Mattavelli L, Sala G, Trem-olizzo L, Schapira AHV, Martinuzzi A, Carelli V & Ferrarese C (2004) Brain127, 2183–2192]. . | iFEBS Journal Antioxidant defences in cybrids harboring mtDNA mutations associated with Leber s hereditary optic neuropathy Maura Floreani1 Eleonora Napoli1 2 Andrea Martinuzzi2 Giorgia Pantano2 Valentina De Riva2 Roberta Trevisan1 2 Elena Bisetto1 2 Lucia Valente1 Valerio Carelli3 and Federica Dabbeni-Sala1 1 Department of Pharmacology and Anesthesiology Pharmacology Section University of Padova Italy 2 E. Medea Scientific Institute Conegliano Research Centre Conegliano Italy 3 Department of NeurologicalSciences University of Bologna Italy Keywords antioxidant enzymes galactose medium GSH and GSSG Leber s hereditary optic neuropathy LHON cybrids oxidative stress Correspondence A. Martinuzzi E. Medea Scientific Institute Conegliano Research Centre via Costa Alta 37 31015 Conegliano TV Italy E-mail Received 2 October 2004 revised 13 December 2004 accepted 22 December 2004 doi Oxidative stress and imbalance between free radical generation and detoxification may play a pivotal role in the pathogenesis of Leber s hereditary optic neuropathy LHON . Mitochondria carrying the homoplasmic 11778 ND4 3460 ND1 and 14484 ND6 mtDNA point mutations associated with LHON were used to generate osteosarcoma-derived cybrids. Enhanced mitochondrial production of reactive oxygen species has recently been demonstrated in these cybrids Beretta S Mattavelli L Sala G Trem-olizzo L Schapira AHV Martinuzzi A Carelli V Ferrarese C 2004 Brain 127 2183-2192 . The aim of this study was to characterize the antioxidant defences of these LHON-affected cells. The activities of glutathione peroxidase GPx glutathione reductase GR superoxide dismutases SOD and catalase and the amounts of glutathione GSH and oxidized glutathione GSSG were measured in cybrids cultured both in glucose-rich medium and galactose-rich medium. The latter is known to cause oxidative stress and to trigger apoptotic death in these cells. In spite of reduced SOD .

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